| Clinical features in patients with severe Alpha-1 antitrypsin deficiency due to rare genotypes. | Ferrarotti I, Piloni D, Filosa A, Ottaviani S, Barzon V, Balderacchi AM, Corda L, Seebacher C, Magni S, Mariani F, Baderna P, Confalonieri P, Iannacci L, Mancinelli S, Putignano P, Albera C, Stella GM, Monti MC, Corsico AG | 2025-12-31 | Alpha-1-antitrypsin, SERPINA1, lung function, orphan disease, rare mutations | Pulmonology |
| Transcatheter treatment of perimembranous ventricular septal defects: challenges, controversies, and a paradigm shift. | Haddad RN | 2025-05-09 | Bioresorbable, device closure, heart block, perimembranous, transcatheter interventions, ventricular septal defect | Future cardiology |
| A novel hemizygous nonsense variant in DOCK11 causes systemic inflammation and immunodeficiency. | Elsayed A, von Hardenberg S, Atschekzei F, Siek P, Witte T, Sogkas G, Ringshausen FC | 2025-04-22 | Actinopathy, Autoinflammation, Bronchiectasis, DOCK11, DOCK11 deficiency, IgG subclass deficiency, Immunodeficiency, Inborn errors of immunity | Clinical immunology (Orlando, Fla.) |
| Ca2+ Cycling Alteration in a Porcine Model of Right Ventricular Dysfunction. | Antigny F, Luo R, Perrier R, Masson B, Fadel G, Ruffenach G, Saint-Martin Willer A, Akamkam A, Grynblat J, Jaïs X, Le Pavec J, Dang Van S, Brunet D, Lefebvre F, Gérard G, Domenichini S, Boët A, Guihaire J, Gomez AM, Montani D, Benitah JP, Humbert M, Mercier O, Sabourin J | 2025-04-18 | animals, heart failure, humans, sarcoplasmic reticulum, swine | Circulation. Heart failure |
| Pulmonary arterial hypertension and cancer: exploring their resemblance as channelopathies. | Antigny F, Crottès D, Vandier C, Capuano V, Guéguinou M | 2025-04-01 | calcium channels, channelopathy, contraction, invasion, migration, potassium channels, proliferation | Trends in molecular medicine |
| [Physiotherapy in Primary Ciliary Dyskinesia]. | Krämer C, Flock J, Borges-Lüke B, Raidt J, Ringshausen FC | 2025-04-01 | | Pneumologie (Stuttgart, Germany) |
| Respiratory Effectiveness Group Position Statement: Inhaler Choice: Balancing Personalized Health Care and Environmental Responsibility. | Usmani OS, Al-Ahmad M, Allan K, Anzueto A, Braido F, Canonica GW, Lambert T, Lapperre T, Kaplan A, Miravitlles M, Papadopoulos NG, Rhee CK, Roche N, Soriano JB | 2025-03-28 | | Journal of aerosol medicine and pulmonary drug delivery |
| A Systematic Review and Expert Recommendation on the Diagnosis of Pulmonary Hypertension Associated With Lung Disease: A Position Paper of the Spanish Society of Pulmonology and Thoracic Surgery (SEPAR). | Rodríguez-Chiaradía DA, Torres-Castro R, Piccari L, García-Ortega A, Perez-Peñate GM, de Miguel-Díez J, Perez-Rojo R, Cano-Pumarega I, Mora-Cuesta V, Blanco I, Figueira-Gonçalves JM, Del Pozo R, López-Meseguer M, Martínez-Meñaca A, Tenes A, Molina-Molina M, Barberà JA | 2025-03-28 | Chronic lung disease, Early diagnosis, Pulmonary vascular disease, Screening | Archivos de bronconeumologia |
| A Unique Combination of Heterozygous CFTR Gene Variants in a Person with Cystic Fibrosis and Mycobacterium abscessus Infection. | Lemson A, Bosteels C, van Ingen J, Reijers M, Westra D, Hoefsloot W | 2025-03-28 | CFTR modulation therapy, Cystic fibrosis, Mycobacterium abscessus infection, Organoids | Respiration; international review of thoracic diseases |
| Impact of A Multidisciplinary Team Discussion for Genetic Lung Fibrosis. | Franco G, Ba I, Nathan N, Guerin C, Lassus A, Kannengiesser C, Froidure A, Manali E, Bunel V, Bonniaud P, Bouvry D, Debray MP, Juge PA, Epaud R, Louvrier C, Plessier A, de Fontbrune FS, Wémeau-Stervinou L, Adam SM, Chabrol A, Maurac A, Savale L, Montani D, Raynal C, Konyukh M, Mageau A, Crestani B, Cottin V, de Becdelièvre A, Borie R | 2025-03-26 | genetic disorder, interstitial pulmonary fibrosis, multidisciplinary discussion, surfactant related genes, telomerase related genes | Respirology (Carlton, Vic.) |
| Adenine base editing with engineered virus-like particles rescues the CFTR mutation G542X in patient-derived intestinal organoids. | Nicosia L, Pranke I, Latorre RV, Murray JB, Lonetti L, Cavusoglu-Doran K, Dreano E, Costello JP, Carroll M, Melotti P, Sorio C, Sermet-Gaudelus I, Scallan MF, Harrison PT | 2025-03-21 | Cellular therapy, Clinical genetics, Genetic engineering, Health sciences | iScience |
| International liver transplantation society practice guideline update on portopulmonary hypertension. | DuBrock HM, Savale L, Sitbon O, Raevens S, Kawut SM, Fallon MB, Heimbach JK, Chadha RM, Crespo G, Ramsay MA, Krowka MJ | 2025-03-18 | | Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society |
| Comparison of Tricuspid Annular Plane Systolic Excursion to Pulmonary Artery Systolic Pressure Ratio Measured by Transthoracic Echocardiography and Right Heart Catheterization in Pulmonary Arterial Hypertension: Prognostic Implications. | Gutiérrez-Ortiz E, López-Guarch CJ, Jiménez JFD, Coronel ML, Martín de Miguel I, Biscotti Rodil B, Duarte Torres J, Segura de la Cal T, Velázquez Martín MT, Arribas Ynsaurriaga F, Cruz-Utrilla A, Escribano-Subías P | 2025-03-14 | TAPSE/PASP ratio, pulmonary arterial hypertension, right heart catheterization, transthoracic echocardiography, ventricular–arterial coupling | Journal of clinical medicine |
| Pregnancy-related issues in rare and low-prevalence diseases: results of ERN transversal working group on pregnancy and family planning survey. | Zucchi D, Marinello D, Tani C, Fulvio G, Aguilera S, Benachi A, Biller R, Blanco I, Borgards P, Boiteux MC, Brandi ML, Costafreda E, Fonseca JE, Fredi M, Iotova V, Louisse S, Nalli C, Onali M, Power B, Rousset-Jablonski C, Sturz D, Tincani A, Vieira A, Capela S, Dan D, De Backer J, de Die-Smulders C, Dufke A, Artzner EL, Limongelli G, Lorenz B, Papenthin W, Pascau MJ, Raidt J, Ray-Coquard I, Rimmer R, Röhl C, Schneider H, Yap T, Talarico R, Mosca M | 2025-03-10 | Pregnancy-related issues, Rare diseases, Unmet needs | Orphanet journal of rare diseases |
| Total lung capacity is predictive of disease severity and survival in systemic sclerosis: A longitudinal analysis in 2347 patients from the French National Cohort Study. | Chaigne B, Bense A, Aubourg F, Agard C, Allanore Y, Berezne A, Pugnet G, Hachulla E, Cottin V, Hot A, Dunogue B, Kanagaratnam A, Palat S, Lescoat A, Berthier S, Chatelus E, Rivière S, Launay D, Truchetet ME, Dinh-Xuan AT, Mouthon L | 2025-03-03 | Disease severity, Forced vital capacity, Interstitial lung disease, Lung fibrosis, Prognosis, Survival, Systemic sclerosis, Total lung capacity | Journal of autoimmunity |
| Comparing methods to measure the dispersion of breathing parameters during exercise testing: A simulation study based on real-life parameters from patients with dysfunctional breathing. | Genecand L, Jaksic C, Desponds R, Simian G, Guerreiro I, Thorens S, Altarelli M, Frésard I, Cantero C, Bringard A, Beurnier A, Laveneziana P, Montani D, Bergeron A, Lador F, Bridevaux PO | 2025-03-01 | abnormal breathing pattern, cardio‐pulmonary exercise testing, dispersion, dysfunctional breathing, simulations | Physiological reports |
| The moving target of progressive pulmonary fibrosis: insights for future clinical trials. | Cottin V, Mismetti V, Valenzuela C, Oldham J | 2025-03-01 | | The European respiratory journal |
| Haemodynamic spectrum in heritable pulmonary arterial hypertension: a continuum from pre-capillary to combined pulmonary hypertension-case series. | Martín de Miguel I, Cruz Utrilla A, Segura de La Cal T, Sarnago Cebada F, Velázquez Martín M, Jiménez López-Guarch C, Arribas Ynsaurriaga F, Escribano Subías P | 2025-03-01 | Cardiovascular comorbidities, Case series, Diastolic dysfunction, Exercise right heart catheterization, Heritable pulmonary arterial hypertension, Post-capillary pulmonary hypertension, Pre-capillary pulmonary hypertension | European heart journal. Case reports |
| MedBot vs RealDoc: efficacy of large language modeling in physician-patient communication for rare diseases. | Weber MT, Noll R, Marchl A, Facchinello C, Grünewaldt A, Hügel C, Musleh K, Wagner TOF, Storf H, Schaaf J | 2025-02-25 | artificial intelligence, health communication, medical informatics, natural language processing, rare diseases | Journal of the American Medical Informatics Association : JAMIA |
| Future treatment paradigms in pulmonary arterial hypertension: a personal view from physicians, health authorities, and patients. | Rahaghi FF, Humbert M, Hoeper MM, White RJ, Frantz RP, Hassoun PM, Hemnes AR, Kawut SM, McLaughlin VV, Meszaros G, Mol PGM, Nathan SD, Psotka MA, Rahaghi FN, Sitbon O, Stockbridge N, Weatherald J, Zannad F, Sahay S | 2025-02-20 | | The Lancet. Respiratory medicine |
| A proposal to involve people living with rare and complex conditions in the development of clinical practice guidelines. | Granja-Dominguez A, Martin-Gomez C, Ortigoza-Escobar JD, Rodriguez-Lopez R, Gonzalez-Bermudez L, Dantone S, Pavanello S, Blasco-Amaro JA | 2025-02-19 | Clinical practice guideline, Guideline development, Patient advisory group, Patient involvement, Patient-centric, Rare disease | Patient education and counseling |
| Sex-Specific Differences in Chronic Thromboembolic Pulmonary Hypertension Treated with Balloon Pulmonary Angioplasty. | Kurzyna P, Witowicz A, Kędzierski P, Florczyk M, Banaszkiewicz M, Szwed P, Piłka M, Gąsecka A, Pietrasik A, Torbicki A, Kurzyna M, Darocha S | 2025-01-29 | balloon pulmonary angioplasty, chronic thromboembolic pulmonary hypertension, pulmonary hypertension, sex-specific differences | Journal of clinical medicine |
| Automated AI-based image analysis for quantification and prediction of interstitial lung disease in systemic sclerosis patients. | Guiot J, Henket M, Gester F, André B, Ernst B, Frix AN, Smeets D, Van Eyndhoven S, Antoniou K, Conemans L, Gote-Schniering J, Slabbynck H, Kreuter M, Sellares J, Tomos I, Yang G, Ribbens C, Louis R, Cottin V, Tomassetti S, Smith V, Walsh SLF | 2025-01-24 | Artificial intelligence, Computed tomography, Interstitial lung disease, Pulmonary function tests, Systemic sclerosis | Respiratory research |
| Dual RNA sequencing of a co-culture model of Pseudomonas aeruginosa and human 2D upper airway organoids. | Pleguezuelos-Manzano C, Beenker WAG, van Son GJF, Begthel H, Amatngalim GD, Beekman JM, Clevers H, den Hertog J | 2025-01-17 | Pseudomonas aeruginosa, 2D co-culture, Airway organoids, Dual RNA-sequencing, Infection model, Quorum sensing | Scientific reports |
| Immune-Boosting and Antiviral Effects of Antioxidants in COVID-19 Pneumonia: A Therapeutic Perspective. | Sanduzzi Zamparelli S, Sanduzzi Zamparelli A, Bocchino M | 2025-01-16 | COVID-19 pneumonia, N-acetylcysteine, antioxidants, melatonin, nitric oxide, ozone, polyphenols, vitamin C, vitamin D | Life (Basel, Switzerland) |
| The Importance of Dose Escalation in the Treatment of Pulmonary Arterial Hypertension with Treprostinil. | Kędzierski P, Banaszkiewicz M, Florczyk M, Piłka M, Mańczak R, Wieteska-Miłek M, Szwed P, Kasperowicz K, Wrona K, Darocha S, Torbicki A, Kurzyna M | 2025-01-13 | dose escalation, prostacyclin, pulmonary arterial hypertension, respond to treatment, treprostinil | Biomedicines |
| Continued Treatment with Nintedanib in Patients with Progressive Pulmonary Fibrosis: Data from INBUILD-ON. | Wuyts WA, Bonella F, Chaudhuri N, Varone F, Antin-Ozerkis D, Song JW, Miede C, Dumistracel M, Coeck C, Cottin V | 2025-01-09 | Clinical trial, Drug tolerance, Interstitial lung disease, Pulmonary function tests, Vital capacity | Lung |
| Comprehensive exploration of unexplained dyspnoea in subjects with normal ejection fraction and low natriuretic peptides. | Berthelot E, Laouar T, Beurnier A, Hrynchynshyn N, Eicher JC, Tartière JM, Jourdain P, Lairez O, Gellen B | 2025-01-09 | HFpEF, NT‐proBNP, diagnosis, dyspnoea, oxygen consumption | ESC heart failure |
| Interstitial Lung Disease Associated with Anti-Ku Antibodies: A Case Series of 19 Patients. | Petitgrand L, Ahmad K, Gamondès D, Diesler R, Fabien N, Gallay L, Fort R, Traclet J, Lestelle F, Chapurlat R, Confavreux CB, Durupt S, Turquier S, Si-Mohamed SA, Coutant F, Cottin V | 2025-01-03 | autoimmunity, connective tissue disease, pulmonary fibrosis | Journal of clinical medicine |
| Exploring the Role of Tertiary Lymphoid Structures Using a Mouse Model of Bacteria-Infected Lungs. | Teillaud JL, Regard L, Martin C, Sibéril S, Burgel PR | 2025-01-01 | B cell, Chronic obstructive pulmonary disease, Cystic fibrosis, Pseudomonas aeruginosa, Staphylococcus aureus, Tertiary lymphoid structure | Methods in molecular biology (Clifton, N.J.) |
| Childhood interstitial lung disease survivors in adulthood: a European collaborative study. | Manali ED, Griese M, Nathan N, Uzunhan Y, Borie R, Michel K, Schwerk N, Fijolek J, Radzikowska E, Chua F, Pabary R, Mogulkoc N, McCarthy C, Kallieri M, Papaioannou AI, Kiper N, Koziar Vasakova M, Lacina L, Molina-Molina M, Torrent-Vernetta A, Tsiligiannis T, Karadag B, Kokosi M, Renzoni EA, Hm van Moorsel C, Campo I, Bendstrup E, Skovhus Prior T, Prasse A, Bonella F, Cottin V, Diesler R, Froidure A, Kolilekas L, Fotis L, Douros K, Kaditis AG, Jeny F, Chauveau S, Nunes H, Dahbia A, Mariani F, van der Vis JJ, Groen K, Erdem Eralp E, Gokdemir Y, Kocakaya D, Olgun Yildizeli S, Yalçın E, Emiralioğlu N, Nayir Buyuksahin H, O'Brien H, Karcıoglu O, Can D, Ezircan A, Kartal Ozturk G, Ocal N, Yuksel H, Narin Tongal S, Safrankova M, Kourtesi K, Louvrier C, Kannengiesser C, Fabre A, Legendre M, Crestani B, Pohunek P, Bush A, Papiris SA | 2024-12-05 | | The European respiratory journal |
| Evaluation of efficacy and safety of rituximab in patients with progressive interstitial lung disease (ILD) with inflammatory component (EvER-ILD2): A multicentre double-blind placebo-controlled randomized trial. | Ferreira M, Bejan-Angoulvant T, Marchand-Adam S, Mousset E, Mureau E, Jouneau S, Nunes H, Montani D, Chenivesse C, Cadranel J, Bonniaud P, Crestani B, Cottin V, Caille A | 2024-11-28 | Hypersensibility pneumonia, Ild, Progressive pulmonary fibrosis, Rituximab | Respiratory medicine and research |
| Prognostic relevance of Exercise Pulmonary Hypertension: Results of the multi-center PEX-NET Clinical Research Collaboration. | Kovacs G, Humbert M, Avian A, Lewis GD, Ulrich S, Vonk Noordegraaf A, Souza R, Galiè N, Malhotra R, Saxer S, Grünig E, Egenlauf B, Ewert R, Heine A, Tedford RJ, Houston BA, Kasperowicz K, Kurzyna M, Rosenkranz S, Herkenrath S, Barbera JA, Blanco I, Oliveira RKF, Andersen M, Savale L, Systrom D, Maron BA, Tello K, Condliffe R, Mak S, Baratto C, Hsu S, D'Alto M, McCabe C, Herve P, Olschewski H | 2024-11-27 | | The European respiratory journal |
| Pulmonary lymphangiomatosis: insights into an ultra-rare disease. | Polke M, Polke N, Piel S, Brunnemer E, Wälscher J, Buschulte K, Warth A, Heussel CP, Eichinger M, Frankenstein L, Eichhorn M, Miliauskas S, Herth FJF, Kreuter M | 2024-11-26 | | Respiratory research |
| One year of ETI reduces lung bacterial colonisation in adults with cystic fibrosis. | Mianowski L, Doléans-Jordheim A, Barraud L, Rabilloud M, Richard M, Josserand RN, Durieu I, Reynaud Q | 2024-11-26 | Haemophilus influenzae, Pseudomonas aeruginosa, Staphylococcus aureus, Cystic fibrosis, Elexacaftor-tezacaftor-ivacaftor, Nontuberculous mycobacteria | Scientific reports |
| Pulmonary Hypertension: From Therapeutic Nihilism to Multiple Therapeutic Interventions. | McLaughlin VV, Humbert M | 2024-11-19 | history, hypertension, pulmonary, therapeutics | Circulation |
| Navigating Surgical Risks: The Impact of BMI Outliers on Pulmonary Endarterectomy Outcomes. | Mercier O | 2024-11-15 | | The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation |
| Pulmonary hypertension in patients carrying FLNA loss-of-function variants. | Stourm L, Grynblat J, Savale L, Lacoste-Palasset T, Jaïs X, Coulet F, Levy M, Meyrignac O, Ghigna MR, Cottin V, Sitbon O, Bonnet D, Goupil F, Humbert M, Gagnadoux F, Montani D | 2024-11-07 | | The European respiratory journal |
| [Management of adult bronchiectasis - Consensus-based Guidelines for the German Respiratory Society (DGP) e. V. (AWMF registration number 020-030)]. | Ringshausen FC, Baumann I, de Roux A, Dettmer S, Diel R, Eichinger M, Ewig S, Flick H, Hanitsch L, Hillmann T, Koczulla R, Köhler M, Koitschev A, Kugler C, Nüßlein T, Ott SR, Pink I, Pletz M, Rohde G, Sedlacek L, Slevogt H, Sommerwerck U, Sutharsan S, von Weihe S, Welte T, Wilken M, Rademacher J, Mertsch P | 2024-11-01 | | Pneumologie (Stuttgart, Germany) |
| Pulmonary fibrosis in patients with autoimmune pulmonary alveolar proteinosis: a retrospective nationwide cohort study. | Guirriec Y, Luque-Paz D, Bernard G, Mabo A, Kerjouan M, Ménard C, Monnier D, Nunes H, Uzunhan Y, Reynaud-Gaubert M, Bermudez J, Borie R, Crestani B, Traclet J, Wémeau-Stervinou L, Chenivesse C, Gomez E, Prévot G, Bourdin A, Bondue B, Bergeron A, Cottin V, Lederlin M, Jouneau S | 2024-11-01 | | ERJ open research |
| Transcatheter correction of a rare combined anomalous pulmonary and systemic venous return in an adult. | Albenque G, Batteux C, Hascoët S | 2024-10-29 | | European heart journal |
| Targeting neutrophil serine proteases in bronchiectasis. | Chalmers JD, Mall MA, Chotirmall SH, O'Donnell AE, Flume PA, Hasegawa N, Ringshausen FC, Watz H, Xu JF, Shteinberg M, McShane PJ | 2024-10-28 | | The European respiratory journal |
| Long-Term Lung Function and Pseudomonas aeruginosa Infection in Genotyped Primary Ciliary Dyskinesia. | Holgersen MG, Marthin JK, Raidt J, Qvist T, Johansen HK, Omran H, Nielsen KG | 2024-10-24 | | Annals of the American Thoracic Society |
| Development of a Nomogram for Genetic Risk of PAH. | Cruz-Utrilla A, Vargas-Ursúa F, Fernández-Villar A, Escribano-Subías P | 2024-10-22 | | Archivos de bronconeumologia |
| Longitudinal evaluation of individuals with severe alpha-1 antitrypsin deficiency (Pi*ZZ genotype). | Fromme M, Payancé A, Mandorfer M, Thorhauge KH, Pons M, Miravitlles M, Stolk J, van Hoek B, Stirnimann G, Frankova S, Sperl J, Kremer AE, Burbaum B, Schrader C, Kadioglu A, Walkenhaus M, Schneider CV, Klebingat F, Balcar L, Kappe NN, Schaefer B, Chorostowska-Wynimko J, Aigner E, Gensluckner S, Striedl P, Roger P, Ryan J, Roche S, Vögelin M, Ala A, Bantel H, Verbeek J, Mariño Z, Praktiknjo M, Gevers TJG, Reuken PA, Berg T, George J, Demir M, Bruns T, Trautwein C, Zoller H, Trauner M, Genesca J, Griffiths WJ, Clark V, Krag A, Turner AM, McElvaney NG, Strnad P | 2024-10-14 | Fibroscan, SERPINA1, lung emphysema | Gastroenterology |
| Quantitative CT-analysis of over aerated lung tissue and correlation with fibrosis extent in patients with idiopathic pulmonary fibrosis. | Tonelli R, Smit MR, Castaniere I, Casa GD, Andrisani D, Gozzi F, Bruzzi G, Cerri S, Samarelli AV, Raineri G, Spagnolo P, Rizzoni R, Ball L, Paulus F, Bos LDJ, Clini E, Marchioni A | 2024-10-05 | Interstitial lung disease, Lung hyperinflation, Mechanotransduction, Pulmonary fibrosis, Residual volume, Total lung capacity, Usual interstitial pneumonia | Respiratory research |
| [Pulmonary Embolism]. | Barca-Hernando M, García-Ortega A, Martínez-Meñaca A, Ramírez-Martín MP, Rivas-Guerrero A, Tenes A | 2024-10-01 | Anticoagulation, Biomarkers, Chronic thromboembolic disease, Chronic thromboembolic pulmonary hypertension, Pulmonary embolism | Open respiratory archives |
| Kidney effects of triple CFTR modulator therapy in people with cystic fibrosis. | Gabai P, Novel-Catin E, Reynaud Q, Nove-Josserand R, Pelletier S, Fouque D, Koppe L, Durieu I | 2024-10-01 | cystic fibrosis, elexacaftor/tezacaftor/ivacaftor, glomerular filtration rate, risk of urolithiasis, secondary hyperaldosteronism | Clinical kidney journal |
| [Highlights 57th SEPAR Congress]. | Martínez Meñaca A, García Moyano M, Sánchez-Salcedo P, Cascón-Hernández J, Sante Diciolla N, Muñoz-Ezquerre M, Barbero Herranz E, Alonso Pérez T | 2024-10-01 | Asthma, Chronic obstructive pulmonary disease, Interstitial lung diseases, Pulmonary vascular diseases, Respiratory physiotherapy | Open respiratory archives |
| Safety and efficacy of rodatristat ethyl for the treatment of pulmonary arterial hypertension (ELEVATE-2): a dose-ranging, randomised, multicentre, phase 2b trial. | Sitbon O, Skride A, Feldman J, Sahay S, Shlobin OA, McLaughlin V, Ghofrani HA, Langleben D, Parsley E, D'Souza G, Marmon T, Kamau-Kelley W, Jones R, Grewal R, Wring S, Palacios M, Naik H, Denning J, Lazarus HM, Humbert M | 2024-09-19 | | The Lancet. Respiratory medicine |
| The IgA-pIgR System Is Dysregulated in Idiopathic Pulmonary Fibrosis. | Planté-Bordeneuve T, Bertrand Y, Lecocq M, Hoton D, Fillée C, Lacroix V, Rondelet B, Wuyts W, Bouzin C, Pilette C, Froidure A | 2024-09-15 | | American journal of respiratory and critical care medicine |
| Rituximab and mycophenolate mofetil in interstitial lung disease (EVER-ILD): one-year follow up results of a randomized controlled trial. | Mansy L, Caille A, Reynaud-Gaubert M, Bermudez J, Bonniaud P, Borie R, Brillet PY, Cadranel J, Court-Fortune I, Crestani B, Debray MP, Duprez M, Guillaumot A, Hirschi-Santelmo S, Israel-Biet D, Jouneau S, Juvin K, Kerjouan M, Mankikian J, Marquette CH, Naccache JM, Nunes H, Plantier L, Prevot G, Quetant S, Traclet J, Valentin V, Uzunhan Y, Wémeau-Stervinou L, Cottin V, Marchand-Adam S | 2024-09-04 | | The European respiratory journal |
| Effect of Sotatercept on Circulating Proteomics in Pulmonary Arterial Hypertension. | Savale L, Tu L, Normand C, Boucly A, Sitbon O, Montani D, Olsson KM, Park DH, Fuge J, Kamp JC, Humbert M, Hoeper MM, Guignabert C | 2024-09-03 | | The European respiratory journal |
| The Seventh World Symposium on Pulmonary Hypertension: our journey to Barcelona. | Humbert M, Galiè N, Rubin LJ, Simonneau G, McLaughlin VV | 2024-09-02 | | The European respiratory journal |
| Evidence from recent clinical trials in fibrotic interstitial lung diseases. | Cottin V, Valenzuela C | 2024-09-01 | | Current opinion in pulmonary medicine |
| Epidemiology of supplemental oxygen in patients with pulmonary hypertension. | Torres-Castro R, Hinojosa W, Martínez-Meñaca A, Sala Llinas E, Jiménez Arjona J, Rueda Soriano J, Aurtenetxe A, Barberà JA, Escribano-Subías P, Blanco I | 2024-08-29 | chronic thromboembolic pulmonary hypertension, incidence, long‐term oxygen therapy, prevalence, pulmonary arterial hypertension | Respirology (Carlton, Vic.) |
| Exploring the patient perspective in pulmonary hypertension. | Ford HJ, Brunetti C, Ferrari P, Meszaros G, Moles VM, Skaara H, Torbicki A, Gibbs JSR | 2024-08-29 | | The European respiratory journal |
| Pathology and pathobiology of pulmonary hypertension: current insights and future directions. | Guignabert C, Aman J, Bonnet S, Dorfmüller P, Olschewski AJ, Pullamsetti S, Rabinovitch M, Schermuly RT, Humbert M, Stenmark KR | 2024-08-29 | | The European respiratory journal |
| Pulmonary hypertension associated with lung diseases. | Shlobin OA, Adir Y, Barbera JA, Cottin V, Harari S, Jutant EM, Pepke-Zaba J, Ghofrani HA, Channick R | 2024-08-29 | | The European respiratory journal |
| A 24/7 Pilot Remote Emergency Multidisciplinary Discussion For Rapidly Progressive Interstitial Lung Disease: A 2-Years Experience. | Bay P, Pineton de Chambrun M, Allenbach Y, Le Pavec J, Picard C, Zuber B, Bunel V, Hervier B, Meyer A, Miyara M, Brillet PY, Boussouar S, Declercq C, Tandjaoui-Lambiotte Y, Nunes H, Cottin V, Hachulla E, Uzunhan Y | 2024-08-26 | MDA5, intensive care unit, interstitial lung diseases, lung transplantation, multidisciplinary team discussion, rapidly progressive interstitial lung disease | Chest |
| Intimacy and sexual life of females with cystic fibrosis. | Simonnet Bisson F, Fauvernier M, Belleguic C, Boucher ID, Grenet D, Hamidfar R, Hubert D, Macey J, Murris-Espin M, Porzio M, Ramel S, Reynaud Q, Reynaud-Gaubert M, Burgel PR | 2024-08-24 | Cystic fibrosis, Females, Sex life, Sexual disorders, Sexual quality of life | Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society |
| Assessment and monitoring of lung disease in patients with severe alpha 1 antitrypsin deficiency: a european delphi consensus of the EARCO group. | Miravitlles M, Turner AM, Sucena M, Mornex JF, Greulich T, Wencker M, McElvaney NG | 2024-08-19 | Alpha 1 antitrypsin deficiency, Augmentation therapy, Computed tomography, Delphi consensus survey, Disease management, Plethysmography, Spirometry | Respiratory research |
| Quantitative imaging methods in combined pulmonary fibrosis and emphysema. | Wang JM, Araki T, Cottin V, Han MK, Oldham JM | 2024-08-16 | chronic obstructive pulmonary disease, combined pulmonary fibrosis and emphysema, computed tomography, emphysema, imaging, interstitial lung disease, pulmonary fibrosis, quantitative | Chest |
| Bexotegrast in Patients with Idiopathic Pulmonary Fibrosis: The INTEGRIS-IPF Clinical Trial. | Lancaster L, Cottin V, Ramaswamy M, Wuyts WA, Jenkins RG, Scholand MB, Kreuter M, Valenzuela C, Ryerson CJ, Goldin J, Kim GHJ, Jurek M, Decaris M, Clark A, Turner S, Barnes CN, Achneck HE, Cosgrove GP, Lefebvre ÉA, Flaherty KR | 2024-08-15 | IPF, efficacy, fibrotic disease, safety | American journal of respiratory and critical care medicine |
| Genetics and Genomics of Pulmonary Fibrosis: Charting the Molecular Landscape and Shaping Precision Medicine. | Adegunsoye A, Kropski JA, Behr J, Blackwell TS, Corte TJ, Cottin V, Glanville AR, Glassberg MK, Griese M, Hunninghake GM, Johannson KA, Keane MP, Kim JS, Kolb M, Maher TM, Oldham JM, Podolanczuk AJ, Rosas IO, Martinez FJ, Noth I, Schwartz DA | 2024-08-15 | MUC5B promoter variant, genetic variants, interstitial lung diseases, precision medicine, pulmonary fibrosis | American journal of respiratory and critical care medicine |
| The Role of Inflammation and Fibrosis in Interstitial Lung Disease Treatment Decisions. | Behr J, Salisbury ML, Walsh SLF, Podolanczuk AJ, Hariri LP, Hunninghake GM, Kolb M, Ryerson CJ, Cottin V, Beasley MB, Corte T, Glanville AR, Adegunsoye A, Hogaboam C, Wuyts WA, Noth I, Oldham JM, Richeldi L, Raghu G, Wells AU | 2024-08-15 | | American journal of respiratory and critical care medicine |
| The expanded French compassionate programme for elexacaftor-tezacaftor-ivacaftor use in people with cystic fibrosis without a F508del CFTR variant: a real-world study. | Burgel PR, Sermet-Gaudelus I, Girodon E, Durieu I, Houdouin V, Audousset C, Macey J, Grenet D, Porzio M, Murris-Espin M, Reix P, Baravalle M, Belleguic C, Mely L, Verhille J, Weiss L, Reynaud-Gaubert M, Mittaine M, Hamidfar R, Ramel S, Cosson L, Douvry B, Danner-Boucher I, Foucaud P, Roy C, Burnet E, Raynal C, Audrezet MP, Da Silva J, Martin C | 2024-08-13 | | The Lancet. Respiratory medicine |
| Clinical and echocardiographic characterization of patients hospitalized for severe tricuspid valve regurgitation: a single tertiary-care center experience with 2-year follow-up. | Piasecki A, Tomaniak M, Gumiężna K, Kurzyna P, Bednarek A, Skulimowska J, Pedzich E, Kapłon-Cieślicka A, Rdzanek A, Ścisło P | 2024-08-08 | | Polish archives of internal medicine |
| Evaluation of treatment response with serial CT in patients with non-tuberculous mycobacterial pulmonary disease. | Dettmer S, Heiß-Neumann M, Wege S, Maske H, Ringshausen FC, Joean O, Theissig N, Ewen R, Wacker F, Rademacher J | 2024-08-01 | Computed tomography, Nontuberculous mycobacteria, Nontuberculous mycobacterial pulmonary disease, Treatment response | European radiology |
| Comparative Effectiveness of Sotatercept and Approved Add-On Pulmonary Arterial Hypertension Therapies: A Systematic Review and Network Meta-Analysis. | Pitre T, Desai K, Mah J, Zeraatkar D, Humbert M | 2024-08-01 | PAH, meta-analysis, sotatercept | Annals of the American Thoracic Society |
| Ceftolozane/Tazobactam for the Treatment of Adults With Cystic Fibrosis: Results From a French Prospective Cohort Study. | Burgel PR, Bourge X, Mackosso C, Parquin F | 2024-08-01 | carbapenem, cystic fibrosis, gram-negative bacteria, multidrug resistance, real-world evidence | Open forum infectious diseases |
| Alcohol consumption and liver phenotype of individuals with alpha-1 antitrypsin deficiency. | Fromme M, Schneider CV, Guldiken N, Amzou S, Luo Y, Pons M, Genesca J, Miravitlles M, Thorhauge KH, Mandorfer M, Waern J, Schneider KM, Sperl J, Frankova S, Bartel M, Zimmer H, Zorn M, Krag A, Turner A, Trautwein C, Strnad P | 2024-07-19 | SERPINA1, FibroScan, Pi*Z, alcohol, liver cirrhosis, liver fibrosis | Liver international : official journal of the International Association for the Study of the Liver |
| Sex-Differences in Alpha-1 Antitrypsin Deficiency: Data From the EARCO Registry. | Ersöz H, Torres-Durán M, Turner AM, Tanash H, García CR, Corsico AG, López-Campos JL, Miravitlles M, Clarenbach CF, Chapman KR, Pérez JMH, Guimarães C, Bartošovská E, Greulich T, Barrecheguren M, Koczulla AR, Höger P, Rivera AO, Herth F, Trudzinski FC | 2024-07-09 | Alpha1-antitrypsin, Alpha1-antitrypsin deficiency, Chronic obstructive pulmonary disease, Gender | Archivos de bronconeumologia |
| Initial combination therapy with macitentan and tadalafil in patients with pulmonary arterial hypertension, with and without cardiac comorbidities. | McLaughlin VV, Sitbon O, Chin KM, Galiè N, Hoeper MM, Kiely DG, MacDonald G, Martin N, Mathai SC, Peacock A, Tawakol A, Torbicki A, Noordegraaf AV, Rosenkranz S | 2024-07-05 | Cardiac comorbidities, Initial double combination therapy, Macitentan, Pulmonary arterial hypertension, Risk stratification, Tadalafil | European journal of heart failure |
| Phenotypes in Pulmonary Hypertension. | Weatherald J, Hemnes AR, Maron BA, Mielniczuk LM, Gerges C, Price LC, Hoeper MM, Humbert M | 2024-07-04 | | The European respiratory journal |
| Pulmonary Arterial Wedge Pressure in Healthy Subjects - a Meta-Analysis. | Zeder K, Avian A, Mak S, Giannakoulas G, Kawut SM, Maron BA, Humbert M, Olschewski H, Kovacs G | 2024-07-04 | | The European respiratory journal |
| EMBARCing on a new era for bronchiectasis: a review series for the Seventh World Bronchiectasis Conference. | Chalmers JD, Goeminne PC, Ringshausen FC | 2024-07-01 | | European respiratory review : an official journal of the European Respiratory Society |
| Growing from common ground: nontuberculous mycobacteria and bronchiectasis. | Van Braeckel E, Bosteels C | 2024-07-01 | | European respiratory review : an official journal of the European Respiratory Society |
| Multisystemic Effects of Elexacaftor-Tezacaftor-Ivacaftor in Adults with Cystic Fibrosis and Advanced Lung Disease. | Burgel PR, Paillasseur JL, Durieu I, Reynaud-Gaubert M, Hamidfar R, Murris-Espin M, Danner-Boucher I, Chiron R, Leroy S, Douvry B, Grenet D, Mely L, Ramel S, Montcouquiol S, Burnet E, Ouaalaya EH, Sogni P, Da Silva J, Martin C | 2024-07-01 | cystic fibrosis transmembrane conductance regulator modulators, real-word evidence, severe cystic fibrosis liver disease | Annals of the American Thoracic Society |
| A systematic literature review of the clinical and socioeconomic burden of bronchiectasis. | Chalmers JD, Mall MA, McShane PJ, Nielsen KG, Shteinberg M, Sullivan SD, Chotirmall SH | 2024-07-01 | | European respiratory review : an official journal of the European Respiratory Society |
| New drugs, new challenges in cystic fibrosis care. | Fajac I, Burgel PR, Martin C | 2024-07-01 | | European respiratory review : an official journal of the European Respiratory Society |
| Anxiety and Depression in Patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension after the Removal of COVID-19 Pandemic Restrictions. | Wieteska-Miłek M, Witowicz A, Szmit S, Florczyk M, Peller M, Dzienisiewicz M, Kurzyna M | 2024-06-17 | HADS scale, anxiety, chronic thromboembolic pulmonary hypertension, depression, fear of COVID-19, pandemic, pulmonary arterial hypertension | Journal of clinical medicine |
| Primary Ciliary Dyskinesia in Adult Bronchiectasis - Data from the German Bronchiectasis Registry PROGNOSIS. | Ewen R, Pink I, Sutharsan S, Aries SP, Grünewaldt A, Shoemark A, Sommerwerck U, Staar BO, Wege S, Mertsch P, Rademacher J, Ringshausen FC | 2024-06-14 | Bronchiectasis, Kartagener syndrome, phenotype, primary ciliary dyskinesia, registries | Chest |
| Correlation Between Tacrolimus Levels in Blood Samples Obtained from Central Catheter and Peripheral Venipuncture in Lung Transplant Patients (Ven-Cat Study). | Diego-Fernández V, García-Saiz MDM, Llorente-Cantalapiedra A, Arquero-González JA, Bermúdez-García MV, Catalán-Ramírez MM, Cornejo-Callejo P, Nuria de Pedro-Simón M, Díez-Pérez MJ, Gandarillas-Ruiz P, Hernández-González F, Herranz-Arenillas P, Laso-Boada MJ, Medina-Gonzalo G, Rodríguez-López A, Ruiz-Antolín M, Álamo-Ibañez M, Cos-Cossio MLÁ, Lavín-Alconero L, Mora-Cuesta VM | 2024-06-11 | | Therapeutic drug monitoring |
| Phenotypic and pathomechanistic overlap between tapasin and TAP deficiencies. | Elsayed A, von Hardenberg S, Atschekzei F, Graalmann T, Jänke C, Witte T, Ringshausen FC, Sogkas G | 2024-06-10 | MHC class I, MHC class I deficiency, TAPBP, bronchiectasis, immunodeficiency, inborn errors of immunity, tapasin | The Journal of allergy and clinical immunology |
| A randomised phase II study of extended pleurectomy/decortication (e-PD) preceded or followed by chemotherapy in patients (pts) with early-stage pleural mesothelioma (PM): EORTC 1205. | Raskin J, Surmont V, Maat APWM, Yahia M, Burgers SA, Pretzenbacher Y, De Ryck F, Cornelissen R, Klomp HM, Oliveira A, Aerts J, Baas P, Marreaud S, Van Meerbeeck JP, Van Schil PEY | 2024-06-06 | | The European respiratory journal |
| Chronic Thromboembolic Pulmonary Hypertension: When Thrombus Mandates Definite Diagnosis to Occur in the Operating Room. | Martín de Miguel I, López-Gude MJ, Martínez Meñaca A, Revilla Ostolaza Y, Arribas Ynsaurriaga F, Escribano Subías P | 2024-06-05 | acenocumarol, endarterectomy, magnetic resonance imaging, pulmonary hypertension thrombosis, tomography | Circulation. Cardiovascular imaging |
| [Primary ciliary dyskinesia]. | Raidt J, Staar BO, Omran H, Ringshausen FC | 2024-06-01 | Hydrocephalus, Infertility, Kartagener syndrome, Mucociliary clearance, Situs inversus | Innere Medizin (Heidelberg, Germany) |
| Primum non nocere, secundum cavere, tertium sanare: lessons on the management of pulmonary hypertension associated with COPD. | Kovacs G, Humbert M | 2024-06-01 | | The European respiratory journal |
| Centralized intestinal organoid generation is a feasible and safe approach for personalized medicine as demonstrated in the HIT-CF Europe Organoid Study. | Bierlaagh MC, van Mourik P, Vonk AM, Pott J, Muilwijk D, Berkers G, Aalbers BL, Vleggaar FP, Michel S, Boj SF, Vries RGJ, Beekman JM, van der Ent CK | 2024-05-18 | Forceps biopsy, Intestinal organoids, Personalized medicine, Rectal biopsy, Suction biopsy | Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society |
| The impact of time from ILD diagnosis to referral to the transplant center on the probability of inclusion in the transplant waiting list. | Mora-Cuesta VM, Martínez-Meñaca A, González-Fernández Á, Iturbe-Fernández D, Tello-Mena S, Izquierdo-Cuervo S, Fernández-Rozas S, Alonso-Lecue P, Cifrián-Martínez JM | 2024-05-11 | Interstitial lung disease, Lung transplantation, Rehabilitation, Transplant center | Heart & lung : the journal of critical care |
| Interstitial Lung Disease Associated with Systemic Sclerosis. | Mismetti V, Si-Mohamed S, Cottin V | 2024-05-07 | | Seminars in respiratory and critical care medicine |
| Considerations for the use of inhaled antibiotics for Pseudomonas aeruginosa in people with cystic fibrosis receiving CFTR modulator therapy. | Burgel PR, Ballmann M, Drevinek P, Heijerman H, Jung A, Mainz JG, Peckham D, Plant BJ, Schwarz C, Taccetti G, Smyth A | 2024-05-03 | Bacterial Infection, Bronchiectasis, Cystic Fibrosis, Respiratory Infection | BMJ open respiratory research |
| Editing Physicians' Responses Using GPT-4 for Academic Research. | Weber MT, Schaaf J, Storf H, Wagner TOF, Berger A, Noll R | 2024-04-26 | Artificial Intelligence, Data Anonymisation, Medical Informatics, Natural Language Processing | Studies in health technology and informatics |
| Prime editing functionally corrects cystic fibrosis-causing CFTR mutations in human organoids and airway epithelial cells. | Bulcaen M, Kortleven P, Liu RB, Maule G, Dreano E, Kelly M, Ensinck MM, Thierie S, Smits M, Ciciani M, Hatton A, Chevalier B, Ramalho AS, Casadevall I Solvas X, Debyser Z, Vermeulen F, Gijsbers R, Sermet-Gaudelus I, Cereseto A, Carlon MS | 2024-04-24 | CRISPR, DETECTOR, cystic fibrosis, gene editing, human nasal epithelial cells, machine learning, patient-derived organoids, prime editing | Cell reports. Medicine |
| Safety and Outcomes of Inferior Vena Cava Filter Placement in Oncology Patients: A Single-Centre Experience. | Kurzyna P, Banaszkiewicz M, Florczyk M, Kępski J, Piłka M, Kędzierski P, Mańczak R, Szwed P, Kasperowicz K, Wrona K, Doroszewski G, Torbicki A, Kurzyna M, Szmit S, Darocha S | 2024-04-19 | cancer-associated thrombosis, deep venous thrombosis, inferior vena cava filter, oncology, pulmonary embolism, venous thromboembolism | Cancers |
| Ultra-low dose chest CT for the diagnosis of pulmonary arteriovenous malformation in patients with hereditary hemorrhagic telangiectasia. | Delpon JE, Greffier J, Lacombe H, Barbe A, Bouin M, De Oliveira F, Mansuy A, Delagrange L, Fargeton AE, Beregi JP, Cottin V, Dupuis-Girod S, Si-Mohamed SA | 2024-04-10 | Hereditary hemorrhagic, Image enhancement, Multidetector computed tomography, Pulmonary arteriovenous malformation, Telangiectasia, Ultra-low dose protocol | Diagnostic and interventional imaging |
| Phenotypes and outcome of diffuse pulmonary non-amyloid light chain deposition disease. | Lestelle F, Beigelman C, Rotzinger D, Si-Mohamed S, Nasser M, Wemeau L, Hirschi S, Prevot G, Roux A, Bunel V, Gomez E, Sohier L, Pradier HM, Gaubert MR, Gondouin A, Lazor R, Glerant JC, Bejui FT, Colombat M, Cottin V | 2024-04-10 | Bronchiectasis, Light chain deposition disease, Lung cysts, Lung transplantation | Respiratory research |
| High risk of lung cancer in surfactant-related gene variant carriers. | Brudon A, Legendre M, Mageau A, Bermudez J, Bonniaud P, Bouvry D, Cadranel J, Cazes A, Crestani B, Dégot T, Delestrain C, Diesler R, Epaud R, Philippot Q, Theou-Anton N, Kannengiesser C, Ba I, Debray MP, Fanen P, Manali E, Papiris S, Nathan N, Amselem S, Gondouin A, Guillaumot A, Andréjak C, Jouneau S, Beltramo G, Uzunhan Y, Galodé F, Westeel V, Mehdaoui A, Hirschi S, Leroy S, Marchand-Adam S, Nunes H, Picard C, Prevot G, Reynaud-Gaubert M, De Vuyst P, Wemeau L, Defossez G, Zalcman G, Cottin V, Borie R | 2024-04-04 | | The European respiratory journal |
| Combined pulmonary fibrosis and emphysema syndrome: the age of majority. | Cottin V | 2024-04-01 | | The European respiratory journal |
| PREFUL MRI for Monitoring Perfusion and Ventilation Changes after Elexacaftor-Tezacaftor-Ivacaftor Therapy for Cystic Fibrosis: A Feasibility Study. | Dohna M, Voskrebenzev A, Klimeš F, Kaireit TF, Glandorf J, Pallenberg ST, Ringshausen FC, Hansen G, Renz DM, Wacker F, Dittrich AM, Vogel-Claussen J | 2024-04-01 | CFTR, Comparative Studies, Cystic Fibrosis, Elexacaftor-Tezacaftor-Ivacaftor Therapy, Fourier Decomposition, Free‐Breathing Proton MRI, Functional MRI, Kaftrio, Lung, MR–Functional Imaging, Modulator Therapy, PREFUL, Pediatrics, Perfusion, Pulmonary, Pulmonary MRI | Radiology. Cardiothoracic imaging |
| MERIT reloaded-what has changed in CTEPH management since 2017? | Torbicki A, Kurzyna M | 2024-04-01 | | The Lancet. Respiratory medicine |
| Pulmonary vascular phenotype identified in patients with GDF2 (BMP9) or BMP10 variants: an international multicentre study. | Grynblat J, Bogaard HJ, Eyries M, Meyrignac O, Savale L, Jaïs X, Ghigna MR, Celant L, Meijboom L, Houweling AC, Levy M, Antigny F, Chaouat A, Cottin V, Guignabert C, Coulet F, Sitbon O, Bonnet D, Humbert M, Montani D | 2024-04-01 | | The European respiratory journal |
| Quality of Life and the Cardiopulmonary Exercise Test in Pulmonary Arterial Hypertension Patients. | Martínez-Meñaca A, Mora-Cuesta VM, Iturbe-Fernández D, Sáinz-Ezquerra Belmonte B, Fernández-Cavia G, Gallardo-Ruiz MJ | 2024-04-01 | | Archivos de bronconeumologia |
| Similarities and differences of interstitial lung disease associated with pathogenic variants in SFTPC and ABCA3 in adults. | Diesler R, Legendre M, Si-Mohamed S, Brillet PY, Wemeau L, Manali ED, Gagnadoux F, Hirschi S, Lorillon G, Reynaud-Gaubert M, Bironneau V, Blanchard E, Bourdin A, Dominique S, Justet A, Macey J, Marchand-Adam S, Morisse-Pradier H, Nunes H, Papiris SA, Traclet J, Traore I, Crestani B, Amselem S, Nathan N, Borie R, Cottin V | 2024-04-01 | ABCA3, SFTPC, adults, fibrosing interstitial lung disease, interstitial lung disease, surfactant | Respirology (Carlton, Vic.) |
| Comment on the article titled "Impact of sex on outcome after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension" published by J. Chan et al. | Cruz-Utrilla A, López-Gude MJ, Velázquez M, Arribas-Ynsaurriaga F, Escribano-Subias P | 2024-04-01 | | The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation |
| Clinical course of suspected familial and sporadic idiopathic pulmonary fibrosis: Data from the PROOF-Next registry. | Froidure A, Bondue B, Dahlqvist C, Guiot J, Gusbin N, Wirtz G, Brusselle G, Strens D, Slabbynck H, Wuyts WA | 2024-04-01 | clinical respiratory medicine, interstitial lung disease, pulmonary fibrosis | Respirology (Carlton, Vic.) |
| The Initial Experience of Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension in Latvia. | Krigere A, Kalejs VR, Kaulins R, Rudzitis A, Bondare L, Sablinskis M, Lejnieks A, Kigitovica D, Kurzyna M, Skride A | 2024-03-30 | Latvia, balloon pulmonary angioplasty, chronic thromboembolic pulmonary hypertension | Medicina (Kaunas, Lithuania) |
| Pulmonary Hypertension in Connective Tissue Diseases Other than Systemic Sclerosis. | Budhram B, Weatherald J, Humbert M | 2024-03-18 | | Seminars in respiratory and critical care medicine |
| Chronic interstitial lung disease associated with systemic lupus erythematosus: A multicentric study of 89 cases. | Deneuville L, Mageau A, Debray MP, Sacre K, Costedoat-Chalumeau N, Hachulla E, Uzunhan Y, Le Tallec E, Cadranel J, Marchand Adam S, Montani D, Rémi-Jardin M, Reynaud-Gaubert M, Prevot G, Beltramo G, Crestani B, Cottin V, Borie R | 2024-03-17 | connective-tissue disease, disease progression, interstitial lung disease, lupus, non-specific interstitial pneumonia, pulmonary fibrosis, survival | Respirology (Carlton, Vic.) |
| Pulmonary fibrosis is uncommon in primary Sjögren disease. | Si-Mohamed SA, Cottin V | 2024-03-15 | Computed tomography, Diffuse parenchymal lung disease, Interstitial lung disease, Primary Sjögren disease, Pulmonary fibrosis | Diagnostic and interventional imaging |
| Health care resource utilization preceding death or lung transplantation in people with cystic fibrosis: HCRU before transplant or death in cystic fibrosis. | Guyot E, Reynaud Q, Belhassen M, Bérard M, Dehillotte C, Lemonnier L, Viprey M, Van Ganse E, Burgel PR, Durieu I | 2024-03-12 | Claims data, Costs, Cystic fibrosis, Healthcare resource utilization, National registry, Transplantation | Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society |
| Invasive Pulmonary Aspergillosis with Exclusive Pleural Involvement in a Lung Transplant Recipient. | García-Martín S, Mora-Cuesta VM, Naranjo-Gozalo S, Jiménez-Velasco JJ, Roiz-Mesones MP, Fernández-Lobo V | 2024-03-11 | Aspergilllus fumigatus, clinical outcomes, infection, invasive pulmonary aspergillosis, lung transplantation, pneumology | Progress in transplantation (Aliso Viejo, Calif.) |
| Treatments for pulmonary arterial hypertension: navigating through a network of choices. | Pitre T, Weatherald J, Humbert M | 2024-03-05 | | European heart journal |
| Corrigendum to "Expert management of congenital portosystemic shunts and their complications" [JHEP Reports 6 (2024)]. | McLin VA, Franchi-Abella S, Brütsch T, Bahadori A, Casotti V, de Ville de Goyet J, Dumery G, Gonzales E, Guérin F, Hascoet S, Heaton N, Kuhlmann B, Lador F, Lambert V, Marra P, Plessier DA, Quaglia A, Rougemont AL, Savale L, Sarma MS, Sitbon O, Superina RA, Uchida H, van Albada M, Johannes van der Doef HP, Vilgrain V, Wacker J, Zwaveling-Soonawala N, Debray D, Wildhaber BE | 2024-03-01 | | JHEP reports : innovation in hepatology |
| Lumacaftor/Ivacaftor Population Pharmacokinetics in Pediatric Patients with Cystic Fibrosis: A First Step Toward Personalized Therapy. | Bouazza N, Urien S, Foissac F, Choupeaux L, Lui G, Froelicher Bournaud L, Rouillon S, Zheng Y, Bardin E, Stremler N, Bessaci K, Bihouee T, Coirier-Duet E, Marguet C, Deneuville E, Laurans M, Reix P, Gerardin M, Mittaine M, Epaud R, Thumerelle C, Weiss L, Berthaud R, Semeraro M, Treluyer JM, Benaboud S, Sermet-Gaudelus I | 2024-03-01 | | Clinical pharmacokinetics |
| C-reactive protein as a candidate biomarker in fibrotic interstitial lung disease. | Cottin V, Valenzuela C | 2024-03-01 | C-reactive protein, blood biomarker, idiopathic pulmonary fibrosis, interstitial lung diseases, progressive pulmonary fibrosis, treatable trait | Respirology (Carlton, Vic.) |
| Effect of Macitentan in Pulmonary Arterial Hypertension and the Relationship Between Echocardiography and cMRI Variables: REPAIR Echocardiography Sub-study Results. | Torbicki A, Channick R, Galiè N, Kiely DG, Moceri P, Peacock A, Swift AJ, Tawakol A, Vonk Noordegraaf A, Flores D, Martin N, Rosenkranz S | 2024-03-01 | Agreement, Cardiac magnetic resonance imaging, Correlation, Echocardiography, Macitentan, Pulmonary arterial hypertension | Cardiology and therapy |
| Impact of Air Pollution and MUC5B Genotype on Survival in Idiopathic Pulmonary Fibrosis. | Sesé L, Borie R, Kannengiesser C, Cottin V, Israel-Biet D, Crestani B, Cadranel J, Chenivesse C, Boubaya M, Valeyre D, Annesi-Maesano I, Nunes H | 2024-03-01 | | Annals of the American Thoracic Society |
| Risk Stratification in Pulmonary Veno-Occlusive Disease. | Cruz-Utrilla A, Pérez-Olivares C, Luna-López R, Jiménez López-Guarch C, Bedate P, Martínez Meñaca A, López Meseguer M, Escribano-Subias P | 2024-02-24 | | Archivos de bronconeumologia |
| Zinpentraxin Alfa for Idiopathic Pulmonary Fibrosis: The Randomized Phase III STARSCAPE Trial. | Richeldi L, Schiffman C, Behr J, Inoue Y, Corte T, Cottin V, Jenkins RG, Nathan SD, Raghu G, Walsh SLF, Jayia PK, Kamath N, Martinez FJ | 2024-02-14 | Antifibrotic medication, Forced vital capacity (FVC), Futility analysis, Interstitial lung disease, Linear mixed-effects model | American journal of respiratory and critical care medicine |
| Reversal of cylindrical bronchial dilatations in a subset of adults with cystic fibrosis treated with elexacaftor-tezacaftor-ivacaftor. | Cazier P, Chassagnon G, Dhote T, Da Silva J, Kanaan R, Honore I, Carlier N, Revel MP, Canniff E, Martin C, Burgel PR | 2024-02-08 | | The European respiratory journal |
| Role of Oxygen Starvation in Right Ventricular Decompensation and Failure in Pulmonary Arterial Hypertension. | Oknińska M, Zajda K, Zambrowska Z, Grzanka M, Paterek A, Mackiewicz U, Szczylik C, Kurzyna M, Piekiełko-Witkowska A, Torbicki A, Kieda C, Mączewski M | 2024-02-01 | angiogenesis, hypertrophy, oxygen deficiency, pulmonary arterial hypertension, right ventricular failure | JACC. Heart failure |
| A formula for predicting emphysema extent in combined idiopathic pulmonary fibrosis and emphysema. | Wells AU, Jacob J, Sverzellati N, Cross G, Barnett J, De Lauretis A, Antoniou K, Weycker D, Atwood M, Kirchgaessler KU, Cottin V | 2024-01-18 | Clinical trial cohort, Interstitial lung disease, Pulmonary function test, Radiology, Real-world cohort | Respiratory research |
| Publisher Correction: Pulmonary hypertension. | Mocumbi A, Humbert M, Saxena A, Jing ZC, Sliwa K, Thienemann F, Archer SL, Stewart S | 2024-01-17 | | Nature reviews. Disease primers |
| Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues. | Burgel PR, Southern KW, Addy C, Battezzati A, Berry C, Bouchara JP, Brokaar E, Brown W, Azevedo P, Durieu I, Ekkelenkamp M, Finlayson F, Forton J, Gardecki J, Hodkova P, Hong G, Lowdon J, Madge S, Martin C, McKone E, Munck A, Ooi CY, Perrem L, Piper A, Prayle A, Ratjen F, Rosenfeld M, Sanders DB, Schwarz C, Taccetti G, Wainwright C, West NE, Wilschanski M, Bevan A, Castellani C, Drevinek P, Gartner S, Gramegna A, Lammertyn E, Landau EEC, Plant BJ, Smyth AR, van Koningsbruggen-Rietschel S, Middleton PG | 2024-01-16 | Breathing, CFRD, Complications, Cystic fibrosis, End of life, Liver, Lung infection, Mental health, Pulmonary, Transplant | Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society |
| Pulmonary hypertension. | Mocumbi A, Humbert M, Saxena A, Jing ZC, Sliwa K, Thienemann F, Archer SL, Stewart S | 2024-01-04 | | Nature reviews. Disease primers |
| Mepolizumab and benralizumab in patients with severe asthma and a history of eosinophilic granulomatosis with polyangiitis. | Desaintjean C, Ahmad K, Traclet J, Gerfaud-Valentin M, Durel CA, Glerant JC, Hot A, Lestelle F, Mainbourg S, Nasser M, Seve P, Turquier S, Devouassoux G, Cottin V | 2024-01-01 | Interleukin-5, asthma, corticosteroids, eosinophil, granulomatosis, vasculitis | Frontiers in medicine |
| Abnormal functional lymphoid tolerance and enhanced myeloid exocytosis are characteristics of resting and stimulated PBMCs in cystic fibrosis patients. | Gaudin C, Ghinnagow R, Lemaire F, Villeret B, Sermet-Gaudelus I, Sallenave JM | 2024-01-01 | PBMCs, Pseudomonas aeruginosa, cystic fibrosis, low-density neutrophils, lymphocyte, proteases, tolerance | Frontiers in immunology |
| Simplified risk stratification based on cardiopulmonary exercise test: A Spanish two-center experience. | Martínez-Meñaca A, Cruz-Utrilla A, Mora-Cuesta VM, Luna-López R, Segura-de la Cal T, Flox-Camacho Á, Alonso-Lecue P, Escribano-Subias P, Cifrián-Martínez JM | 2024-01-01 | 6 min walking test, cardiopulmonary exercise test, pulmonary arterial hypertension, risk assessment | Pulmonary circulation |
| Gathering real-world compassionate data to expand eligibility for elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis with N1303K or other rare CFTR variants: a viewpoint. | Burgel PR, Sermet-Gaudelus I, Girodon E, Kanaan R, Le Bihan J, Remus N, Ravoninjatovo B, Grenet D, Porzio M, Houdouin V, Le Clainche-Viala L, Durieu I, Nove-Josserand R, Languepin J, Coltey B, Guillaumot A, Audousset C, Chiron R, Weiss L, Fajac I, Da Silva J, Martin C | 2024-01-01 | | The European respiratory journal |
| Expert management of congenital portosystemic shunts and their complications. | McLin VA, Franchi-Abella S, Brütsch T, Bahadori A, Casotti V, de Ville de Goyet J, Dumery G, Gonzales E, Guérin F, Hascoet S, Heaton N, Kuhlmann B, Lador F, Lambert V, Marra P, Plessier A, Quaglia A, Rougemont AL, Savale L, Sarma MS, Sitbon O, Superina RA, Uchida H, van Albada M, Johannes van der Doef HP, Vilgrain V, Wacker J, Zwaveling N, Debray D, Wildhaber BE | 2024-01-01 | adenoma, congenital portosystemic shunt, focal nodular hyperplasia, hepatocellular carcinoma, hyperandrogenism, hyperinsulinism, hypoglycemia, occlusion test, portal pressure, puberty, pulmonary hypertension, β-catenin | JHEP reports : innovation in hepatology |
| A BEAT-PCD consensus statement: a core outcome set for pulmonary disease interventions in primary ciliary dyskinesia. | Kos R, Goutaki M, Kobbernagel HE, Rubbo B, Shoemark A, Aliberti S, Altenburg J, Anagnostopoulou P, Athanazio RA, Beydon N, Dell SD, Emiralioglu N, Ferkol TW, Loebinger MR, Lorent N, Maître B, Marthin J, Morgan LC, Nielsen KG, Ringshausen FC, Shteinberg M, Tiddens HAWM, Maitland-Van der Zee AH, Chalmers JD, Lucas JSA, Haarman EG | 2024-01-01 | | ERJ open research |
| [Genetic diffuse cystic lung disease in adults]. | Diesler R, Ahmad K, Chalabreysse L, Glérant JC, Harzallah I, Touraine R, Si-Mohamed S, Cottin V | 2024-01-01 | Birt-Hogg-Dubé, Birt-Hogg-Dubé syndrome, Diffuse cystic lung disease, Genetic, Génétique, Interstitial lung disease, Lymphangioleiomyomatosis, Lymphangioléiomyomatose, Maladie kystique pulmonaire, Pneumopathie interstitielle diffuse, Sclérose tubéreuse de Bourneville, Tuberous sclerosis complex | Revue des maladies respiratoires |
| The fibroblast activation protein alpha as a biomarker of pulmonary fibrosis. | Lavis P, Garabet A, Cardozo AK, Bondue B | 2024-01-01 | FAP, FAPI, IPF, PPF, biomarker, fibroblast activation protein, fibrosis, progression | Frontiers in medicine |
| Bexotegrast in people with idiopathic pulmonary fibrosis (IPF): a plain language summary of publication of the INTEGRIS-IPF study. | Lancaster L, Cottin V, Ramaswamy M, Wuyts WA, Jenkins RG, Scholand MB, Kreuter M, Valenzuela C, Ryerson CJ, Goldin J, Kim GHJ, Jurek M, Decaris M, Clark A, Turner SM, Barnes CN, Achneck HE, Cosgrove GP, Lefebvre ÉA, Flaherty KR | 2024-01-01 | IPF, efficacy, fibrotic disease, safety | Therapeutic advances in respiratory disease |
| Characteristics and outcomes of patients with LAM receiving sirolimus in France based on real-life data. | Cottin V, Cases A, Bourdin V, Reynaud-Gaubert M, Hirschi S, Kerjouan M, Diesler R, Chardès B, Fiévez S, Assi N, Schmidt A, Denis H, Wémeau-Stervinou L, Uzunhan Y | 2024-01-01 | lung-disease, lymphangioleiomyomatosis, rapamycin, real-life data, sirolimus, survival, tuberous sclerosis complex | Frontiers in medicine |
| Infections in autoimmune pulmonary alveolar proteinosis: a large retrospective cohort. | Mabo A, Borie R, Wemeau-Stervinou L, Uzunhan Y, Gomez E, Prevot G, Reynaud-Gaubert M, Traclet J, Bergot E, Cadranel J, Marchand-Adam S, Bergeron A, Blanchard E, Bondue B, Bonniaud P, Bourdin A, Burgel PR, Hirschi S, Marquette CH, Quétant S, Nunes H, Chenivesse C, Crestani B, Guirriec Y, Monnier D, Ménard C, Tattevin P, Cottin V, Luque Paz D, Jouneau S | 2023-12-15 | GM-CSF autoantibody, bacterial Infection, opportunist lung infections, pulmonary alveolar proteinosis | Thorax |
| The Utility of a Resting Electrocardiogram (ECG-PH Index) in Evaluating the Efficacy of Pulmonary Endarterectomy in Chronic Thromboembolic Pulmonary Hypertension. | Piłka M, Darocha S, Florczyk M, Mańczak R, Banaszkiewicz M, Kędzierski P, Zieliński D, Wróbel K, Torbicki A, Kurzyna M | 2023-12-11 | chronic thromboembolic pulmonary hypertension, electrocardiography, pulmonary endarterectomy, pulmonary hypertension | Journal of clinical medicine |
| Rituximab to treat prolidase deficiency due to a novel pathogenic copy number variation in PEPD. | Atschekzei F, Fedchenko M, Elsayed A, Dubrowinskaja N, Graalmann T, Ringshausen FC, Witte T, Sogkas G | 2023-12-07 | Autoimmune Diseases, Immune System Diseases, Rituximab, Sjogren's Syndrome | RMD open |
| European pathways of care in Alpha-1 Antitrypsin deficiency. | Chorostowska-Wynimko J, Koczulla AR, Sucena M | 2023-12-01 | Alpha-1 antitrypsin deficiency, Diagnosis, European, Survey | Respiratory medicine |
| Long-term safety and efficacy of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis and at least one F508del allele: 144-week interim results from a 192-week open-label extension study. | Daines CL, Tullis E, Costa S, Linnemann RW, Mall MA, McKone EF, Polineni D, Quon BS, Ringshausen FC, Rowe SM, Selvadurai H, Taylor-Cousar JL, Withers NJ, Ahluwalia N, Moskowitz SM, Prieto-Centurion V, Tan YV, Tian S, Weinstock T, Xuan F, Zhang Y, Ramsey B, Griese M | 2023-12-01 | | The European respiratory journal |
| Monographic Issue on Pulmonary Hypertension. | Barberà JA, Humbert M | 2023-12-01 | | Seminars in respiratory and critical care medicine |
| Extracorporeal Life Support in Pulmonary Hypertension: Practical Aspects. | Hoeper MM | 2023-12-01 | | Seminars in respiratory and critical care medicine |
| Pulmonary Hypertension Associated with Left Heart Disease. | Baratto C, Caravita S, Vachiéry JL | 2023-12-01 | | Seminars in respiratory and critical care medicine |
| Updated Hemodynamic Definition and Classification of Pulmonary Hypertension. | Lechartier B, Kularatne M, Jaïs X, Humbert M, Montani D | 2023-12-01 | | Seminars in respiratory and critical care medicine |
| Monographic Issue on Pulmonary Hypertension: Medical and Interventional Treatment for Chronic Thromboembolic Pulmonary Hypertension. | Delcroix M, Belge C, Maleux G, Godinas L | 2023-12-01 | | Seminars in respiratory and critical care medicine |
| The Right Ventricle in Pulmonary Hypertension. | Wessels JN, Celant LR, de Man FS, Vonk Noordegraaf A | 2023-12-01 | | Seminars in respiratory and critical care medicine |
| Management of Pulmonary Hypertension Associated with Chronic Lung Disease. | Blanco I, Hernández-González F, García A, Torres-Castro R, Barberà JA | 2023-12-01 | | Seminars in respiratory and critical care medicine |
| Management of Acutely Decompensated Pulmonary Hypertension. | Savale L, Kularatne M, Roche A, Pichon J, Baron A, Boucly A, Sitbon O, Humbert M | 2023-12-01 | | Seminars in respiratory and critical care medicine |
| Ciliary Ultrastructure Assessed by Transmission Electron Microscopy in Adults with Bronchiectasis and Suspected Primary Ciliary Dyskinesia but Inconclusive Genotype. | Staar BO, Hegermann J, Auber B, Ewen R, von Hardenberg S, Olmer R, Pink I, Rademacher J, Wetzke M, Ringshausen FC | 2023-11-18 | bronchiectasis, genotype–phenotype correlation, primary ciliary dyskinesia, transmission electron microscopy, ultrastructure, whole-exome sequencing | Cells |
| Monitoring of ECFS quality standards for the clinical management of adults with cystic fibrosis. | Gramegna A, Aliberti S, Amorim A, Blasi F, Bourke S, Burgel PR, Diamantea F, Durieu I, Fila L, Moreno RMG, Messore B, Pokojová E, Taccetti G, Verhulst S, Dugac AV, Wege S, Duff A, Southern KW, Castellani C | 2023-11-08 | Adulthood, Cystic fibrosis, Cystic fibrosis centers, Quality standards, Standards of care | Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society |
| Leukocyte telomere length: the dawn of a new era of personalised medicine in fibrotic interstitial lung diseases? | Cottin V, Kolb M | 2023-11-01 | | The European respiratory journal |
| Outcome of lung transplantation for adults with interstitial lung disease associated with genetic disorders of the surfactant system. | Bermudez J, Nathan N, Coiffard B, Roux A, Hirschi S, Degot T, Bunel V, Le Pavec J, Macey J, Le Borgne A, Legendre M, Cottin V, Thomas PA, Borie R, Reynaud-Gaubert M | 2023-11-01 | | ERJ open research |
| Idiopathic pulmonary fibrosis with benign SFTPC variant and pathogenic MARS1 mutations: can't see the forest for the trees! | Castaldo A, Delestrain C, Diesler R, Merveilleux du Vignaux C, Onnee M, Touraine R, Chalabreysse L, Fanen P, Epaud R, Cottin V, De Becdelièvre A | 2023-11-01 | | ERJ open research |
| Viewpoint: activin signalling inhibitors for the treatment of pulmonary arterial hypertension. | Humbert M | 2023-11-01 | | The European respiratory journal |
| Defining the clinical validity of genes reported to cause pulmonary arterial hypertension. | Welch CL, Aldred MA, Balachandar S, Dooijes D, Eichstaedt CA, Gräf S, Houweling AC, Machado RD, Pandya D, Prapa M, Shaukat M, Southgate L, Tenorio-Castano J, Chung WK | 2023-11-01 | Genetics, Genomic medicine, Molecular diagnosis, Pulmonary arterial hypertension | Genetics in medicine : official journal of the American College of Medical Genetics |
| Nonsense mutations accelerate lung disease and decrease survival of cystic fibrosis children. | Orenti A, Pranke I, Faucon C, Varilh J, Hatton A, Golec A, Dehillotte C, Durieu I, Reix P, Burgel PR, Grenet D, Tasset C, Gachelin E, Perisson C, Lepissier A, Dreano E, Tondelier D, Chevalier B, Weiss L, Kiefer S, Laurans M, Chiron R, Lemonnier L, Marguet C, Jung A, Edelman A, Kerem BS, Girodon E, Taulan-Cadars M, Hinzpeter A, Kerem E, Naehrlich L, Sermet-Gaudelus I | 2023-11-01 | Cystic fibrosis (MeSH: DO008550), Cystic fibrosis transmembrane conductance regulator (MeSH: DO19005), Premature termination codon (MeSH: DO:18389) | Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society |
| Bronchial Stenosis After Lung Transplantation From cDCD Donors Using Simultaneous Abdominal Normothermic Regional Perfusion: A Single-center Experience. | Mora-Cuesta VM, Tello-Mena S, Izquierdo-Cuervo S, Iturbe-Fernández D, Sánchez-Moreno L, Ballesteros MA, Alonso-Lecue P, Ortíz-Portal F, Ferrer-Pargada D, Miñambres-García E, Cifrián-Martínez JM, Naranjo-Gozalo S | 2023-11-01 | | Transplantation |
| Exploring intrinsic variability between cultured nasal and bronchial epithelia in cystic fibrosis. | Rodenburg LW, Metzemaekers M, van der Windt IS, Smits SMA, den Hertog-Oosterhoff LA, Kruisselbrink E, Brunsveld JE, Michel S, de Winter-de Groot KM, van der Ent CK, Stadhouders R, Beekman JM, Amatngalim GD | 2023-10-30 | | Scientific reports |
| Seven Additional Patients with SOX17 Related Pulmonary Arterial Hypertension and Review of the Literature. | Gallego-Zazo N, Miranda-Alcaraz L, Cruz-Utrilla A, Del Cerro Marín MJ, Álvarez-Fuente M, Del Mar Rodríguez Vázquez Del Rey M, Guillén Rodríguez I, Becerra-Munoz VM, Moya-Bonora A, Ochoa Parra N, Parra A, Pascual P, Cazalla M, Silván C, Arias P, Valverde D, de Jesús-Pérez V, Lapunzina P, Escribano-Subías P, Tenorio-Castano J | 2023-10-20 | SOX17, cardiovascular disorders, congenital heart disease, genomic medicine, personalized medicine, pulmonary arterial hypertension, whole exome sequencing | Genes |
| Theratyping cystic fibrosis patients to guide elexacaftor/tezacaftor/ivacaftor out-of-label prescription. | Dreano E, Burgel PR, Hatton A, Bouazza N, Chevalier B, Macey J, Leroy S, Durieu I, Weiss L, Grenet D, Stremler N, Ohlmann C, Reix P, Porzio M, Roux Claude P, Rémus N, Douvry B, Montcouquiol S, Cosson L, Mankikian J, Languepin J, Houdouin V, Le Clainche L, Guillaumot A, Pouradier D, Tissot A, Priou P, Mély L, Chedevergne F, Lebourgeois M, Lebihan J, Martin C, Zavala F, Da Silva J, Lemonnier L, Kelly-Aubert M, Golec A, Foucaud P, Marguet C, Edelman A, Hinzpeter A, de Carli P, Girodon E, Sermet-Gaudelus I, Pranke I | 2023-10-01 | | The European respiratory journal |
| Prognostic Stratification and Treatment for Pulmonary Arterial Hypertension. Gaps and Promising Remedies. | Del Pozo R, Cruz-Utrilla A, Escribano-Subias P | 2023-10-01 | | Archivos de bronconeumologia |
| Protocol for generating airway organoids from 2D air liquid interface-differentiated nasal epithelia for use in a functional CFTR assay. | Rodenburg LW, van der Windt IS, Dreyer HHM, Smits SMA, den Hertog-Oosterhoff LA, Aarts EM, Beekman JM, Amatngalim GD | 2023-09-15 | Cell culture, Organoids | STAR protocols |
| The European reference network on rare lung diseases (ERN-LUNG) ambition to offer better lung health for all. | Humbert M, Wagner T | 2023-09-10 | | Presse medicale (Paris, France : 1983) |
| Design of a phase III, double-blind, randomised, placebo-controlled trial of BI 1015550 in patients with progressive pulmonary fibrosis (FIBRONEER-ILD). | Maher TM, Assassi S, Azuma A, Cottin V, Hoffmann-Vold AM, Kreuter M, Oldham JM, Richeldi L, Valenzuela C, Wijsenbeek MS, Coeck C, Schlecker C, Voss F, Wachtlin D, Martinez FJ | 2023-09-01 | Interstitial Fibrosis, Rare lung diseases | BMJ open respiratory research |
| Treatment of pulmonary arterial hypertension: recent progress and a look to the future. | Humbert M, Sitbon O, Guignabert C, Savale L, Boucly A, Gallant-Dewavrin M, McLaughlin V, Hoeper MM, Weatherald J | 2023-09-01 | | The Lancet. Respiratory medicine |
| Interstitial lung disease-associated pulmonary hypertension - what the future holds. | Cottin V, Diesler R, Turquier S, Valenzuela C | 2023-09-01 | | Current opinion in pulmonary medicine |
| Pulmonary alveolar proteinosis - current and future therapeutical strategies. | Jehn LB, Bonella F | 2023-09-01 | | Current opinion in pulmonary medicine |
| Autoantibodies against the NineTeen complex and U5 RNP in systemic sclerosis. | Vulsteke JB, Smith V, Bonroy C, Lenaerts JL, Derua R, Wuyts WA, Blockmans D, De Haes P, Piette Y, De Langhe E, Bossuyt X | 2023-09-01 | | Journal of autoimmunity |
| Comprehensive Clinical Diagnostic Pipelines Reveal New Variants in Alpha-1 Antitrypsin Deficiency. | Ottaviani S, Bartoli G, Carroll TP, Gangemi F, Balderacchi AM, Barzon V, Corino A, Piloni D, McElvaney NG, Corsico AG, Irving JA, Fra A, Ferrarotti I | 2023-09-01 | SERPINA1 rare variants, alpha-1 antitrypsin polymers, pathogenicity predictions, serpinopathies, serpins | American journal of respiratory cell and molecular biology |
| Emerging Role of Balloon Pulmonary Angioplasty in Chronic Thromboembolic Pulmonary Disease-Insights from the 2022 ESC Guidelines. | Banaszkiewicz M, Kurzyna P, Kubikowska N, Mucha M, Rudnik A, Gąsecka A, Pietrasik A, Grabowski M, Jaguszewski MJ, Kasprzyk P, Kędzierski P, Ciećwierz D, Żuk G, Szwed P, Piłka M, Florczyk M, Kurzyna M, Darocha S | 2023-08-16 | balloon pulmonary angioplasty, chronic thromboembolic pulmonary disease, chronic thromboembolic pulmonary hypertension, pulmonary endarterectomy, pulmonary hypertension | Journal of clinical medicine |
| Design of a phase III, double-blind, randomised, placebo-controlled trial of BI 1015550 in patients with idiopathic pulmonary fibrosis (FIBRONEER-IPF). | Richeldi L, Azuma A, Cottin V, Kreuter M, Maher TM, Martinez FJ, Oldham JM, Valenzuela C, Gordat M, Liu Y, Stowasser S, Zoz DF, Wijsenbeek MS | 2023-08-01 | Lung Transplantation | BMJ open respiratory research |
| Alpha1-antitrypsin deficiency: An updated review. | Mornex JF, Traclet J, Guillaud O, Dechomet M, Lombard C, Ruiz M, Revel D, Reix P, Cottin V | 2023-07-29 | | Presse medicale (Paris, France : 1983) |
| Cystic fibrosis. | Fajac I, Burgel PR | 2023-07-27 | Bronchiectasis, CFTR modulators, Cystic fibrosis transmembrane conductance regulator (CFTR), Pseudomonas aeruginosa | Presse medicale (Paris, France : 1983) |
| Optimal treatment of the underlying aetiology is the most effective antimicrobial stewardship for chronic respiratory disease: a lesson learned from cystic fibrosis. | Rademacher J, Martin L, Theloe A, Stahl M, Mall MA, Joean O, Fuge J, Hansen G, Welte T, Schütz K, Ringshausen FC, Dittrich AM | 2023-07-01 | | ERJ open research |
| Diagnosis and management of pulmonary veno-occlusive disease. | Solinas S, Boucly A, Beurnier A, Kularatne M, Grynblat J, Eyries M, Dorfmüller P, Sitbon O, Humbert M, Montani D | 2023-07-01 | EIF2AK4, Pulmonary veno-occlusive disease, chemotherapy, pulmonary edema, pulmonary hypertension, solvents, transplantation | Expert review of respiratory medicine |
| Treatable traits: a comprehensive precision medicine approach in interstitial lung disease. | Khor YH, Cottin V, Holland AE, Inoue Y, McDonald VM, Oldham J, Renzoni EA, Russell AM, Strek ME, Ryerson CJ | 2023-07-01 | | The European respiratory journal |
| Lung Function Decline in Cystic Fibrosis: Impact of Data Availability and Modeling Strategies on Clinical Interpretations. | Szczesniak R, Andrinopoulou ER, Su W, Afonso PM, Burgel PR, Cromwell E, Gecili E, Ghulam E, Goss CH, Mayer-Hamblett N, Keogh RH, Liou TG, Marshall B, Morgan WJ, Ostrenga JS, Pasta DJ, Stanojevic S, Wainwright C, Zhou GC, Fernandez G, Fink AK, Schechter MS | 2023-07-01 | epidemiology, longitudinal analysis, pulmonary function, rate of decline, registry analysis | Annals of the American Thoracic Society |
| CT imaging shows specific pancreatic abnormalities in persons with cystic fibrosis related diabetes. | Alexandre-Heymann L, Puerto M, Martin C, Burnet E, Mosnier-Pudar H, Burgel PR, Larger E | 2023-06-27 | | Scientific reports |
| Inhaled treprostinil for interstitial lung disease-associated pulmonary hypertension: a silver lining on a very dark cloud. | Cottin V, Valenzuela C, Humbert M | 2023-06-01 | | The European respiratory journal |
| Transforming clinical research and science in bronchiectasis: EMBARC3, a European Respiratory Society Clinical Research Collaboration. | Chalmers JD, Aliberti S, Altenburg J, Blasi F, Clarke C, Chotirmall SH, Crichton ML, Dhar R, Goeminne P, Haworth C, Loebinger MR, Lorent N, Polverino E, Ringshausen FC, Shoemark A, Shteinberg M, Sibila O, Spinou A, Welte T | 2023-06-01 | | The European respiratory journal |
| Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis. | Emmi G, Bettiol A, Gelain E, Bajema IM, Berti A, Burns S, Cid MC, Cohen Tervaert JW, Cottin V, Durante E, Holle JU, Mahr AD, Del Pero MM, Marvisi C, Mills J, Moiseev S, Moosig F, Mukhtyar C, Neumann T, Olivotto I, Salvarani C, Seeliger B, Sinico RA, Taillé C, Terrier B, Venhoff N, Bertsias G, Guillevin L, Jayne DRW, Vaglio A | 2023-06-01 | | Nature reviews. Rheumatology |
| French recommendations for the diagnosis and management of lymphangioleiomyomatosis. | Cottin V, Blanchard E, Kerjouan M, Lazor R, Reynaud-Gaubert M, Taille C, Uzunhan Y, Wemeau L, Andrejak C, Baud D, Bonniaud P, Brillet PY, Calender A, Chalabreysse L, Court-Fortune I, Desbaillets NP, Ferretti G, Guillemot A, Hardelin L, Kambouchner M, Leclerc V, Lederlin M, Malinge MC, Mancel A, Marchand-Adam S, Maury JM, Naccache JM, Nasser M, Nunes H, Pagnoux G, Prévot G, Rousset-Jablonski C, Rouviere O, Si-Mohamed S, Touraine R, Traclet J, Turquier S, Vagnarelli S, Ahmad K | 2023-06-01 | Angiomyolipoma, Lymphangioleiomyomatosis, Pneumothorax, Sirolimus, Tuberous sclerosis | Respiratory medicine and research |
| Validating organoid-derived human intestinal monolayers for personalized therapy in cystic fibrosis. | Birimberg-Schwartz L, Ip W, Bartlett C, Avolio J, Vonk AM, Gunawardena T, Du K, Esmaeili M, Beekman JM, Rommens J, Strug L, Bear CE, Moraes TJ, Gonska T | 2023-06-01 | | Life science alliance |
| French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis - 2021 update. Full-length version. | Cottin V, Bonniaud P, Cadranel J, Crestani B, Jouneau S, Marchand-Adam S, Nunes H, Wémeau-Stervinou L, Bergot E, Blanchard E, Borie R, Bourdin A, Chenivesse C, Clément A, Gomez E, Gondouin A, Hirschi S, Lebargy F, Marquette CH, Montani D, Prévot G, Quetant S, Reynaud-Gaubert M, Salaun M, Sanchez O, Trumbic B, Berkani K, Brillet PY, Campana M, Chalabreysse L, Chatté G, Debieuvre D, Ferretti G, Fourrier JM, Just N, Kambouchner M, Legrand B, Le Guillou F, Lhuillier JP, Mehdaoui A, Naccache JM, Paganon C, Rémy-Jardin M, Si-Mohamed S, Terrioux P | 2023-06-01 | Biopsy, Interstitial lung disease, Pulmonary fibrosis, Usual interstitial pneumonia | Respiratory medicine and research |
| Usefulness of genetics for clinical reclassification and refinement of prognostic stratification in pulmonary arterial hypertension. | Cruz-Utrilla A, Gallego-Zazo N, Pérez-Olivares C, Hernández-González I, Bedate P, Martínez Meñaca A, López Meseguer M, Lapunzina P, Pérez Núñez M, Ochoa Parra N, Valverde D, Tenorio-Castaño JA, Escribano-Subias P | 2023-06-01 | Enfermedad venooclusiva pulmonar, Genetics, Genética, Hipertensión arterial pulmonar, Pulmonary arterial hypertension, Pulmonary veno-oclusive disease | Revista espanola de cardiologia (English ed.) |
| Primary Ciliary Dyskinesia Patient-Specific hiPSC-Derived Airway Epithelium in Air-Liquid Interface Culture Recapitulates Disease Specific Phenotypes In Vitro. | von Schledorn L, Puertollano Martín D, Cleve N, Zöllner J, Roth D, Staar BO, Hegermann J, Ringshausen FC, Nawroth J, Martin U, Olmer R | 2023-05-24 | PCD, air-liquid interface culture, human induced pluripotent stem cells | Cells |
| Pseudomonas aeruginosa population genomics among adults with bronchiectasis across Germany. | Rosenboom I, Oguz S, Lüdemann IM, Ringshausen FC, Rademacher J, Sedlacek L, Tümmler B, Cramer N | 2023-05-01 | | ERJ open research |
| The RISE study protocol: resilience impacted by positive stressful events for people with cystic fibrosis. | van der Heijden E, van den Bor RM, van der Ent CK, Nijhof SL, van der Laan SEI | 2023-05-01 | | ERJ open research |
| The U UGA C sequence provides a favorable context to ELX-02 induced CFTR readthrough. | Pranke IM, Varilh J, Hatton A, Faucon C, Girodon E, Dreano E, Chevalier B, Karri S, Reix P, Durieu I, Bidou L, Namy O, Taulan M, Hinzpeter A, Sermet-Gaudelus I | 2023-05-01 | | Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society |
| Long COVID syndrome after SARS-CoV-2 survival in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. | Wieteska-Miłek M, Kuśmierczyk-Droszcz B, Betkier-Lipińska K, Szmit S, Florczyk M, Zieliński P, Hoffman P, Krzesińki P, Kurzyna M | 2023-04-01 | COVID‐19, chronic thromboembolic pulmonary hypertension, long COVID syndrome, pulmonary arterial hypertension, risk of death | Pulmonary circulation |
| Epidemiological trends in nontuberculous mycobacterial infection among people with cystic fibrosis in Germany. | Steindor M, Hafkemeyer S, Ruckes C, Stehling F, Naehrlich L, Ringshausen FC | 2023-04-01 | Cystic fibrosis transmembrane conductance regulator, Epidemiology, Germany, Mycobacterium abscessus, Mycobacterium avium, Registries | International journal of infectious diseases : IJID : official publication of the International Society for Infectious Diseases |
| CFTR Modulators: Current Status and Evolving Knowledge. | Regard L, Martin C, Da Silva J, Burgel PR | 2023-04-01 | | Seminars in respiratory and critical care medicine |
| The world of rare interstitial lung diseases. | Buschulte K, Cottin V, Wijsenbeek M, Kreuter M, Diesler R | 2023-03-31 | | European respiratory review : an official journal of the European Respiratory Society |
| Ordinal outcomes add value to clinical trials - Authors' reply. | Weatherald J, Humbert M | 2023-03-25 | | Lancet (London, England) |
| European Respiratory Society statement on familial pulmonary fibrosis. | Borie R, Kannengiesser C, Antoniou K, Bonella F, Crestani B, Fabre A, Froidure A, Galvin L, Griese M, Grutters JC, Molina-Molina M, Poletti V, Prasse A, Renzoni E, van der Smagt J, van Moorsel CHM | 2023-03-01 | | The European respiratory journal |
| Progressive pulmonary fibrosis: an expert group consensus statement. | Rajan SK, Cottin V, Dhar R, Danoff S, Flaherty KR, Brown KK, Mohan A, Renzoni E, Mohan M, Udwadia Z, Shenoy P, Currow D, Devraj A, Jankharia B, Kulshrestha R, Jones S, Ravaglia C, Quadrelli S, Iyer R, Dhooria S, Kolb M, Wells AU | 2023-03-01 | | The European respiratory journal |
| GLPG1205 for idiopathic pulmonary fibrosis: a phase 2 randomised placebo-controlled trial. | Strambu IR, Seemayer CA, Fagard LMA, Ford PA, Van der Aa TAK, de Haas-Amatsaleh AA, Modgill V, Santermans E, Sondag EN, Helmer EG, Maher TM, Costabel U, Cottin V | 2023-03-01 | | The European respiratory journal |
| PDE5 to keep them alive: The use of phosphodiesterase type-5 inhibitors in severe pulmonary hypertension associated with interstitial lung disease. | Weatherald J, Humbert M | 2023-03-01 | interstitial lung disease, phosphodiesterase type 5 inhibitor, pulmonary fibrosis, pulmonary hypertension, sildenafil, tadalafil, treatment | Respirology (Carlton, Vic.) |
| Lung Dual-Energy CT Perfusion Blood Volume as a Marker of Severity in Chronic Thromboembolic Pulmonary Hypertension. | Si-Mohamed SA, Zumbihl L, Turquier S, Boccalini S, Mornex JF, Douek P, Cottin V, Boussel L | 2023-02-17 | X-ray computed/methods, comparative study, lung, perfusion, tomography | Diagnostics (Basel, Switzerland) |
| The French Compassionate Program of elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis with advanced lung disease and no F508del CFTR variant. | Burgel PR, Sermet-Gaudelus I, Durieu I, Kanaan R, Macey J, Grenet D, Porzio M, Coolen-Allou N, Chiron R, Marguet C, Douvry B, Dufeu N, Danner-Boucher I, Foucaud P, Lemonnier L, Girodon E, Da Silva J, Martin C | 2023-02-16 | | The European respiratory journal |
| Proteomic profiling of sweat in patients with cystic fibrosis provides new insights into epidermal homoeostasis. | Cornet M, Nguyen-Khoa T, Kelly-Aubert M, Jung V, Chedevergne F, Le Bourgeois M, Aoust L, Roger K, Guerrera CI, Sermet-Gaudelus I | 2023-02-01 | | Skin health and disease |
| Identification of new telomere- and telomerase-associated autoantigens in systemic sclerosis. | Vulsteke JB, Smith V, Bonroy C, Derua R, Blockmans D, De Haes P, Vanderschueren S, Lenaerts JL, Claeys KG, Wuyts WA, Verschueren P, Vanhandsaeme G, Piette Y, De Langhe E, Bossuyt X | 2023-02-01 | Autoantibodies, Mass spectrometry, Systemic sclerosis, Telomerase, Telomere | Journal of autoimmunity |
| Towards Translation of PqsR Inverse Agonists: From In Vitro Efficacy Optimization to In Vivo Proof-of-Principle. | Hamed MM, Abdelsamie AS, Rox K, Schütz C, Kany AM, Röhrig T, Schmelz S, Blankenfeldt W, Arce-Rodriguez A, Borrero-de Acuña JM, Jahn D, Rademacher J, Ringshausen FC, Cramer N, Tümmler B, Hirsch AKH, Hartmann RW, Empting M | 2023-02-01 | Pseudomonas aeruginosa, bronchiectasis, in vivo proof-of-concept, pathoblocker, quorum sensing | Advanced science (Weinheim, Baden-Wurttemberg, Germany) |
| Qualitative and quantitative evaluation of computed tomography changes in adults with cystic fibrosis treated with elexacaftor-tezacaftor-ivacaftor: a retrospective observational study. | Dettmer S, Weinheimer O, Sauer-Heilborn A, Lammers O, Wielpütz MO, Fuge J, Welte T, Wacker F, Ringshausen FC | 2023-01-01 | CFTR, computed tomography, cystic fibrosis, elexacaftor-tezacaftor-ivacaftor, quantitative CT, therapy response | Frontiers in pharmacology |
| A practical approach to the 2022 ESC cardio-oncology guidelines: Comments by a team of experts - cardiologists and oncologists. | Leszek P, Klotzka A, Bartuś S, Burchardt P, Czarnecka AM, Długosz-Danecka M, Gierlotka M, Koseła-Paterczyk H, Krawczyk-Ożóg A, Kubiatowski T, Kurzyna M, Maciejczyk A, Mitkowski P, Prejbisz A, Rutkowski P, Sierko E, Sterliński M, Szmit S, Szwiec M, Tajstra M, Tycińska A, Witkowski A, Wojakowski W, Cybulska-Stopa B | 2023-01-01 | | Kardiologia polska |
| Case report: Transected Hickman catheter and its thrombotic occlusion in a patient with idiopathic pulmonary arterial hypertension-can a catheter replacement be avoided? | Sławiński G, Zieleniewicz P, Faran A, Dąbrowska-Kugacka A, Kurzyna M, Kempa M, Daniłowicz-Szymanowicz L, Lewicka E | 2023-01-01 | epoprostenol, hickman and broviac catheters, pulmonary hypertension, repair kit, thrombosis | Frontiers in cardiovascular medicine |
| Spirometric and anthropometric improvements in response to elexacaftor/tezacaftor/ivacaftor depending on age and lung disease severity. | Schütz K, Pallenberg ST, Kontsendorn J, DeLuca D, Sukdolak C, Minso R, Büttner T, Wetzke M, Dopfer C, Sauer-Heilborn A, Ringshausen FC, Junge S, Tümmler B, Hansen G, Dittrich AM | 2023-01-01 | BMI, cystic fibrosis, elexacaftor/tezacaftor/ivacaftor, modulator, pediatric | Frontiers in pharmacology |
| ECMO in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension as a bridge to therapy. | Cruz-Utrilla A, García-Martín EP, Domínguez Pérez L, Ruiz Curiel A, Quezada A, Durante López A, Vicent L, Martín Asenjo R, Hinojosa W, Eixerés A, Forcén Acebal L, Galindo M, Arribas Ynsaurriaga F, Escribano-Subias P, Bueno H | 2023-01-01 | | Kardiologia polska |
| COVID-19 in patients with pulmonary alveolar proteinosis: a European multicentre study. | Papiris SA, Campo I, Mariani F, Kallieri M, Kolilekas L, Papaioannou AI, Gonca Chousein E, Cetinkaya E, Bonella F, Borie R, Kokosi M, Pickworth T, Molina-Molina M, Gasa M, Radzikowska E, Fijolek J, Jouneau S, Gomez E, McCarthy C, Bendstrup E, Piotrowski WJ, Pabary R, Hadchouel A, Coolen-Allou N, Alfaro T, Robalo Cordeiro C, Antonogiannaki EM, Tomos IP, Papakosta D, Kontakiotis T, Panagiotou P, Douros K, Schams A, Lettieri S, Papaevangelou V, Kanaka-Gantenbein C, Karakatsani A, Loukides S, Costabel U, Crestani B, Morgan C, Tazawa R, Bush A, Griese M, Manali ED | 2023-01-01 | | ERJ open research |
| Lung transplantation for end-stage cystic fibrosis before and after the availability of elexacaftor-tezacaftor-ivacaftor, Germany, 2012-2021. | Ringshausen FC, Sauer-Heilborn A, Büttner T, Dittrich AM, Schwerk N, Ius F, Nährlich L, Welte T, Greer M | 2023-01-01 | | The European respiratory journal |
| Normalisation of circulating neutrophil counts after 12 months of elexacaftor-tezacaftor-ivacaftor in patients with advanced cystic fibrosis. | Dhote T, Martin C, Regard L, Pesenti L, Kanaan R, Carlier N, Honoré I, Da Silva J, Witko-Sarsat V, Burgel PR | 2023-01-01 | | The European respiratory journal |
| The Changing Epidemiology of Cystic Fibrosis: The Implications for Adult Care. | Burgel PR, Burnet E, Regard L, Martin C | 2023-01-01 | cystic fibrosis, cystic fibrosis transmembrane conductance regulator, lung transplantation, registry | Chest |
| Crosstalk between cilia and autophagy: implication for human diseases. | Morleo M, Vieira HLA, Pennekamp P, Palma A, Bento-Lopes L, Omran H, Lopes SS, Barral DC, Franco B | 2023-01-01 | Autophagy, cilia, human diseases, lysosome, macroautophagy, mitochondria | Autophagy |
| Redefining Hypo- and Hyper-Responding Phenotypes of CFTR Mutants for Understanding and Therapy. | Hillenaar T, Beekman J, van der Sluijs P, Braakman I | 2022-12-02 | CFTR, hyper-responders, hypo-responding mutants, modulators, protein folding | International journal of molecular sciences |
| European Respiratory Society guidelines on transbronchial lung cryobiopsy in the diagnosis of interstitial lung diseases. | Korevaar DA, Colella S, Fally M, Camuset J, Colby TV, Hagmeyer L, Hetzel J, Maldonado F, Morais A, Ravaglia C, Spijker R, Tomassetti S, Troy LK, Verschakelen JA, Wells AU, Tonia T, Annema JT, Poletti V | 2022-11-01 | | The European respiratory journal |
| Drug Repurposing for Cystic Fibrosis: Identification of Drugs That Induce CFTR-Independent Fluid Secretion in Nasal Organoids. | Rodenburg LW, Delpiano L, Railean V, Centeio R, Pinto MC, Smits SMA, van der Windt IS, van Hugten CFJ, van Beuningen SFB, Rodenburg RNP, van der Ent CK, Amaral MD, Kunzelmann K, Gray MA, Beekman JM, Amatngalim GD | 2022-10-21 | TMEM16A, cystic fibrosis, drug repurposing, nasal organoids, screening assay | International journal of molecular sciences |
| Phenotypes of idiopathic pulmonary arterial hypertension. | Cruz-Utrilla A, Pérez-Olivares C, Martínez-Meñaca A, López-Meseguer M, Escribano-Subias P | 2022-10-01 | | The Lancet. Respiratory medicine |
| Drug-induced interstitial lung disease. | Spagnolo P, Bonniaud P, Rossi G, Sverzellati N, Cottin V | 2022-10-01 | | The European respiratory journal |
| Pharmacological chaperone-rescued cystic fibrosis CFTR-F508del mutant overcomes PRAF2-gated access to endoplasmic reticulum exit sites. | Saha K, Chevalier B, Doly S, Baatallah N, Guilbert T, Pranke I, Scott MGH, Enslen H, Guerrera C, Chuon C, Edelman A, Sermet-Gaudelus I, Hinzpeter A, Marullo S | 2022-09-27 | Arginin-based retention motif, BRET, CFTR, Corrector, ERES, Gatekeeper, Protein export, Sec 24, Sec 31 | Cellular and molecular life sciences : CMLS |
| Clinical Implications of the Genetic Background in Pediatric Pulmonary Arterial Hypertension: Data from the Spanish REHIPED Registry. | Cruz-Utrilla A, Gallego-Zazo N, Tenorio-Castaño JA, Guillén I, Torrent-Vernetta A, Moya-Bonora A, Labrandero C, Rodríguez-Monte MEG, Rodríguez-Ogando A, Rey MDMRVD, Espín J, Plata-Izquierdo B, Álvarez-Fuente M, Moreno-Galdó A, Escribano-Subias P, Marín MJDC | 2022-09-09 | genetics, heritable pulmonary arterial hypertension, pediatric pulmonary hypertension, pulmonary veno-occlusive disease | International journal of molecular sciences |
| [Test Your Knowledge]. | Angelini E, Dettmer S, Goecke T, Länger F, Ringshausen FC | 2022-09-01 | | Pneumologie (Stuttgart, Germany) |
| Clinical impact of TERT somatic mutation in telomerase-related gene mutation carriers after lung transplantation. | Ba I, Kannengiesser C, Mal H, Reynaud-Gaubert M, Cottin V, Hirschi S, Picard C, Borie R | 2022-09-01 | TERT somatic mutation, digital droplet PCR, idiopathic pulmonary fibrosis, lung transplantation, telomerase-related gene | The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation |
| [French practical guidelines for the diagnosis and management of IPF - 2021 update, full version]. | Cottin V, Bonniaud P, Cadranel J, Crestani B, Jouneau S, Marchand-Adam S, Nunes H, Wémeau-Stervinou L, Bergot E, Blanchard E, Borie R, Bourdin A, Chenivesse C, Clément A, Gomez E, Gondouin A, Hirschi S, Lebargy F, Marquette CH, Montani D, Prévot G, Quetant S, Reynaud-Gaubert M, Salaun M, Sanchez O, Trumbic B, Berkani K, Brillet PY, Campana M, Chalabreysse L, Chatté G, Debieuvre D, Ferretti G, Fourrier JM, Just N, Kambouchner M, Legrand B, Le Guillou F, Lhuillier JP, Mehdaoui A, Naccache JM, Paganon C, Rémy-Jardin M, Si-Mohamed S, Terrioux P | 2022-09-01 | Biopsie, Biopsy, Common interstitial lung disease, Fibrose pulmonaire, Interstitial lung disease, Pneumopathie interstitielle commune, Pneumopathie interstitielle diffuse, Pulmonary fibrosis | Revue des maladies respiratoires |
| Differential CFTR-Interactome Proximity Labeling Procedures Identify Enrichment in Multiple SLC Transporters. | Chevalier B, Baatallah N, Najm M, Castanier S, Jung V, Pranke I, Golec A, Stoven V, Marullo S, Antigny F, Guerrera IC, Sermet-Gaudelus I, Edelman A, Hinzpeter A | 2022-08-11 | CFTR, KCNK3, SLC transporters, cystic fibrosis, interactome, proximity labeling | International journal of molecular sciences |
| Measuring cystic fibrosis drug responses in organoids derived from 2D differentiated nasal epithelia. | Amatngalim GD, Rodenburg LW, Aalbers BL, Raeven HH, Aarts EM, Sarhane D, Spelier S, Lefferts JW, Silva IA, Nijenhuis W, Vrendenbarg S, Kruisselbrink E, Brunsveld JE, van Drunen CM, Michel S, de Winter-de Groot KM, Heijerman HG, Kapitein LC, Amaral MD, van der Ent CK, Beekman JM | 2022-08-03 | | Life science alliance |
| Integrating Clinical Probability into the Diagnostic Approach to Idiopathic Pulmonary Fibrosis: An International Working Group Perspective. | Cottin V, Tomassetti S, Valenzuela C, Walsh SLF, Antoniou KM, Bonella F, Brown KK, Collard HR, Corte TJ, Flaherty KR, Johannson KA, Kolb M, Kreuter M, Inoue Y, Jenkins RG, Lee JS, Lynch DA, Maher TM, Martinez FJ, Molina-Molina M, Myers JL, Nathan SD, Poletti V, Quadrelli S, Raghu G, Rajan SK, Ravaglia C, Remy-Jardin M, Renzoni E, Richeldi LK, Spagnolo P, Troy L, Wijsenbeek M, Wilson KC, Wuyts W, Wells AU, Ryerson CJ | 2022-08-01 | | American journal of respiratory and critical care medicine |
| Diversity of approaches in artificial intelligence: an opportunity for discoveries in thoracic imaging. | Chassagnon G, Campredon A, Vakalopoulou M, Burgel PR | 2022-07-01 | | The European respiratory journal |
| Inflammation biomarkers in sputum for clinical trials in cystic fibrosis: current understanding and gaps in knowledge. | Lepissier A, Addy C, Hayes K, Noel S, Bui S, Burgel PR, Dupont L, Eickmeier O, Fayon M, Leal T, Lopes C, Downey DG, Sermet-Gaudelus I | 2022-07-01 | cystic fibrosis, inflammation, sputum biomarkers | Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society |
| Embolization of Recurrent Pulmonary Arteriovenous Malformations by Ethylene Vinyl Alcohol Copolymer (Onyx®) in Hereditary Hemorrhagic Telangiectasia: Safety and Efficacy. | Si-Mohamed SA, Cierco A, Gamondes D, Restier LM, Delagrange L, Cottin V, Dupuis-Girod S, Revel D | 2022-06-30 | Rendu–Osler–Weber disease, arteriovenous malformations, embolization, hereditary hemorrhagic telangiectasia, thorax | Journal of personalized medicine |
| Trial of a Preferential Phosphodiesterase 4B Inhibitor for Idiopathic Pulmonary Fibrosis. | Richeldi L, Azuma A, Cottin V, Hesslinger C, Stowasser S, Valenzuela C, Wijsenbeek MS, Zoz DF, Voss F, Maher TM | 2022-06-09 | | The New England journal of medicine |
| Respiratory management of spinal muscular atrophy type 1 patients treated with Nusinersen. | Menard J, Seferian AM, Fleurence E, Barzic A, Binoche A, Labouret G, Coutier L, Vuillerot C, Bieleu BM, Gomez Garcia de la Banda M, Corvol H, Servais L, Taytard J | 2022-06-01 | children, nocturnal gas exchange, noninvasive ventilation, nusinersen, respiratory management, spinal muscular atrophy type 1 | Pediatric pulmonology |
| CFTR Modulators in People with Cystic Fibrosis: Real-World Evidence in France. | Regard L, Martin C, Burnet E, Da Silva J, Burgel PR | 2022-05-28 | CFTR modulators, cystic fibrosis, elexacaftor, ivacaftor, real-world studies, tezacaftor | Cells |
| The role of cardiopulmonary exercise testing in identifying and monitoring pulmonary veno-oclusive disease: a case report with ING. | Segura de la Cal T, Pérez-Olivares C, Cristo Ropero MJ, Luna López R, Escribano-Subías P | 2022-05-01 | CPET, Cardiopulmonary exercise test, EIF2AK4, Pulmonary arterial hypertension, Pulmonary veno-occlusive disease | European heart journal. Case reports |
| Respiratory failure due to concomitant interstitial lung disease and diaphragmatic involvement in a patient with anti-MDA5 dermatomyositis: a case report. | Grignaschi S, Mongodi S, Alfonsi E, Mojoli F, Vertui V, Zanframundo G, Cavagna L | 2022-05-01 | | Clinical and experimental rheumatology |
| Sustained effectiveness of elexacaftor-tezacaftor-ivacaftor in lung transplant candidates with cystic fibrosis. | Martin C, Reynaud-Gaubert M, Hamidfar R, Durieu I, Murris-Espin M, Danner-Boucher I, Chiron R, Leroy S, Douvry B, Grenet D, Mely L, Ramel S, Montcouquiol S, Lemonnier L, Burnet E, Paillasseur JL, Da Silva J, Burgel PR | 2022-05-01 | | Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society |
| Systemic bis-phosphinic acid derivative restores chloride transport in Cystic Fibrosis mice. | da Cunha MF, Pranke I, Sassi A, Schreiweis C, Moriceau S, Vidovic D, Hatton A, Carlon MS, Creste G, Berhal F, Prestat G, Freund R, Odolczyk N, Jais JP, Gravier-Pelletier C, Zielenkiewicz P, Jullien V, Hinzpeter A, Oury F, Edelman A, Sermet-Gaudelus I | 2022-04-12 | | Scientific reports |
| Safety and tolerability of nintedanib in patients with progressive fibrosing interstitial lung diseases: data from the randomized controlled INBUILD trial. | Cottin V, Martinez FJ, Jenkins RG, Belperio JA, Kitamura H, Molina-Molina M, Tschoepe I, Coeck C, Lievens D, Costabel U | 2022-04-07 | Adverse drug event, Clinical trial, Diarrhea, Patient adherence, Pulmonary fibrosis | Respiratory research |
| Targeted therapy for pulmonary alveolar proteinosis: the time is now. | Bonella F, Borie R | 2022-04-01 | | The European respiratory journal |
| Anetumab ravtansine versus vinorelbine in patients with relapsed, mesothelin-positive malignant pleural mesothelioma (ARCS-M): a randomised, open-label phase 2 trial. | Kindler HL, Novello S, Bearz A, Ceresoli GL, Aerts JGJV, Spicer J, Taylor P, Nackaerts K, Greystoke A, Jennens R, Calabrò L, Burgers JA, Santoro A, Cedrés S, Serwatowski P, Ponce S, Van Meerbeeck JP, Nowak AK, Blumenschein G, Siegel JM, Kasten L, Köchert K, Walter AO, Childs BH, Elbi C, Hassan R, Fennell DA | 2022-04-01 | | The Lancet. Oncology |
| Tobramycin safety and efficacy review article. | Schwarz C, Taccetti G, Burgel PR, Mulrennan S | 2022-04-01 | Cystic fibrosis, FEV(1), Hospitalizations, Pseudomonas aeruginosa, Sputum density, Tobramycin | Respiratory medicine |
| Determinants of survival after lung transplantation in telomerase-related gene mutation carriers: A retrospective cohort. | Phillips-Houlbracq M, Mal H, Cottin V, Gauvain C, Beier F, Sicre de Fontbrune F, Sidali S, Mornex JF, Hirschi S, Roux A, Weisenburger G, Roussel A, Wémeau-Stervinou L, Le Pavec J, Pison C, Marchand Adam S, Froidure A, Lazor R, Naccache JM, Jouneau S, Nunes H, Reynaud-Gaubert M, Le Borgne A, Boutboul D, Ba I, Boileau C, Crestani B, Kannengiesser C, Borie R | 2022-04-01 | clinical research, fibrosis, lung disease, lung transplantation, practice, pulmonology, transplant social worker | American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons |
| European Respiratory Society International Congress 2021: highlights from best-abstract awardees. | Ramakrishnan S, Beaufils F, De Brandt J, Viney K, Bradley C, Cottin V, Hassan M, Cruz J | 2022-03-01 | | Breathe (Sheffield, England) |
| Pulmonary thromboendarterectomy in chronic thromboembolic pulmonary hypertension: the Spanish experience. | López-Gude MJ, Blanco I, Benito-Arnáiz V, Castellà M, Escribano-Subías P, Martin C, Barberà JA, Cortina-Romero JM | 2022-03-01 | Pulmonary thromboendarterectomy, pulmonary hypertension (PH), pulmonary vascular resistance (PVR), residual pulmonary hypertension, operability | Annals of cardiothoracic surgery |
| [French practical guidelines for the diagnosis and management of IPF - 2021 update, short version]. | Cottin V, Bonniaud P, Cadranel J, Crestani B, Jouneau S, Marchand-Adam S, Nunes H, Wémeau-Stervinou L, Bergot E, Blanchard E, Borie R, Bourdin A, Chenivesse C, Clément A, Gomez E, Gondouin A, Hirschi S, Lebargy F, Marquette CH, Montani D, Prévot G, Quetant S, Reynaud-Gaubert M, Salaun M, Sanchez O, Trumbic B, Berkani K, Brillet PY, Campana M, Chalabreysse L, Chatté G, Debieuvre D, Ferretti G, Fourrier JM, Just N, Kambouchner M, Legrand B, Le Guillou F, Lhuillier JP, Mehdaoui A, Naccache JM, Paganon C, Rémy-Jardin M, Si-Mohamed S, Terrioux P | 2022-03-01 | Biopsie, Biopsy, Common interstitial lung disease, Fibrose pulmonaire, Interstitial lung disease, Pneumopathie interstitielle commune, Pneumopathie interstitielle diffuse, Pulmonary fibrosis | Revue des maladies respiratoires |
| Interstitial lung diseases associated with mutations of poly(A)-specific ribonuclease: A multicentre retrospective study. | Philippot Q, Kannengiesser C, Debray MP, Gauvain C, Ba I, Vieri M, Gondouin A, Naccache JM, Reynaud-Gaubert M, Uzunhan Y, Bondue B, Israël-Biet D, Dieudé P, Fourrage C, Lainey E, Manali E, Papiris S, Wemeau L, Hirschi S, Mal H, Nunes H, Schlemmer F, Blanchard E, Beier F, Cottin V, Crestani B, Borie R | 2022-03-01 | PARN, idiopathic interstitial pneumonia, idiopathic pulmonary fibrosis, interstitial lung disease, mutation, poly(A)-specific ribonuclease, telomere homeostasis, variant | Respirology (Carlton, Vic.) |
| Major Decrease in Lung Transplantation for Patients with Cystic Fibrosis in France. | Martin C, Legeai C, Regard L, Cantrelle C, Dorent R, Carlier N, Kerbaul F, Burgel PR | 2022-03-01 | | American journal of respiratory and critical care medicine |
| Natural history of respiratory muscle strength in spinal muscular atrophy: a prospective national cohort study. | Veldhoen ES, Wijngaarde CA, Hulzebos EHJ, Wösten-van Asperen RM, Wadman RI, van Eijk RPA, Asselman FL, Stam M, Otto LAM, Cuppen I, Scheijmans FEV, den Oudenrijn LPV, Bartels B, Gaytant MA, van der Ent CK, van der Pol WL | 2022-02-21 | Lung function, Natural history, Neuromuscular, Respiratory muscle strength, Spinal muscular atrophy | Orphanet journal of rare diseases |
| Pirfenidone in Unclassifiable Interstitial Lung Disease: A Subgroup Analysis by Concomitant Mycophenolate Mofetil and/or Previous Corticosteroid Use. | Kreuter M, Maher TM, Corte TJ, Molina-Molina M, Axmann J, Gilberg F, Kirchgaessler KU, Cottin V | 2022-02-01 | Corticosteroid, Immunomodulator, Mycophenolate mofetil, Pirfenidone, Unclassifiable interstitial lung disease | Advances in therapy |
| Sjögren disease, not Sjögren's: comment on the article by Baer and Hammitt. | Cottin V | 2022-02-01 | | Arthritis & rheumatology (Hoboken, N.J.) |
| Efficacy of Pirfenidone vs. Placebo in Unclassifiable Interstitial Lung Disease, by Surgical Lung Biopsy Status: Data From a post-hoc Analysis. | Molina-Molina M, Kreuter M, Cottin V, Corte TJ, Gilberg F, Kirchgaessler KU, Axmann J, Maher TM | 2022-01-01 | lung function, pirfenidone, post-hoc analysis, surgical lung biopsy, unclassifiable interstitial lung disease | Frontiers in medicine |
| Are anaplastic lymphoma kinase (ALK) and O6-methylguanine-DNA methyltransferase (MGMT) promoter methylation driver biomarkers of pulmonary neuroendocrine tumors (NETs) and carcinomas (NECs)? | Hiddinga B, Zwaenepoel K, Janssens A, Van Meerbeeck J, Pauwels P | 2022-01-01 | MGMT promoter methylation, anaplastic lymphoma kinase, neuroendocrine carcinoma, neuroendocrine tumor, small cell lung cancer | Oncotarget |
| The Burden of Progressive-Fibrosing Interstitial Lung Diseases. | Cottin V, Teague R, Nicholson L, Langham S, Baldwin M | 2022-01-01 | economic burden, epidemiology, humanistic burden, progressive fibrosing ILD, quality of life, survival | Frontiers in medicine |
| Post hoc Analysis of Clinical Outcomes in Placebo- and Pirfenidone-Treated Patients with IPF Stratified by BMI and Weight Loss. | Jouneau S, Crestani B, Thibault R, Lederlin M, Vernhet L, Yang M, Morgenthien E, Kirchgaessler KU, Cottin V | 2022-01-01 | Body composition, Body mass index, Idiopathic pulmonary fibrosis, Interstitial lung disease | Respiration; international review of thoracic diseases |
| A call for evidence in connective tissue diseases-associated interstitial lung disease. | Cottin V, Lega JC, Coury F, Nasser M | 2022-01-01 | Evidence, Guidelines, Interstitial Lung Disease, Pulmonary fibrosis, Recommendations, Sjogren disease | Joint bone spine |
| Real-world assessment of LCI following lumacaftor-ivacaftor initiation in adolescents and adults with cystic fibrosis. | Reix P, Tatopoulos A, Ioan I, Le Bourgeois M, Bui S, Choukroun ML, Bessaci-Kabouya K, Gerardin M, Bokov P, Da Silva J, Paillasseur JL, Burgel PR | 2022-01-01 | CFTR modulators, Cystic fibrosis, Lumacaftor, Multiple breath washout | Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society |
| Impact of a high emergency lung transplantation programme for cystic fibrosis in France: insight from a comparison with Canada. | Coriati A, Sykes J, Lemonnier L, Ma X, Stanojevic S, Dehillotte C, Carlier N, Stephenson AL, Burgel PR | 2022-01-01 | | The European respiratory journal |
| Interstitial pneumonia with autoimmune features: challenges and controversies. | Mackintosh JA, Wells AU, Cottin V, Nicholson AG, Renzoni EA | 2021-12-31 | | European respiratory review : an official journal of the European Respiratory Society |
| Clinical utility of diagnostic biomarkers in malignant pleural mesothelioma: a systematic review and meta-analysis. | Schillebeeckx E, van Meerbeeck JP, Lamote K | 2021-12-31 | | European respiratory review : an official journal of the European Respiratory Society |
| Spectral Photon-Counting CT Technology in Chest Imaging. | Si-Mohamed SA, Miailhes J, Rodesch PA, Boccalini S, Lacombe H, Leitman V, Cottin V, Boussel L, Douek P | 2021-12-09 | computed tomography, diagnostic imaging, lung, photon-counting detectors, thorax | Journal of clinical medicine |
| Using chest CT scan and unsupervised machine learning for predicting and evaluating response to lumacaftor-ivacaftor in people with cystic fibrosis. | Campredon A, Battistella E, Martin C, Durieu I, Mely L, Marguet C, Belleguic C, Murris-Espin M, Chiron R, Fanton A, Bui S, Reynaud-Gaubert M, Reix P, Hoang-Thi TN, Vakalopoulou M, Revel MP, Da Silva J, Burgel PR, Chassagnon G | 2021-11-18 | | The European respiratory journal |
| Long-term amikacin liposome inhalation suspension in cystic fibrosis patients with chronic P. aeruginosa infection. | Bilton D, Fajac I, Pressler T, Clancy JP, Sands D, Minic P, Cipolli M, Galeva I, Solé A, Quittner AL, Jumadilova Z, Ciesielska M, Konstan MW | 2021-11-01 | ALIS, Amikacin, Cystic fibrosis, Liposomal, Pseudomonas aeruginosa | Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society |
| Patient perspectives following initiation of elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis and advanced lung disease. | Martin C, Burnet E, Ronayette-Preira A, de Carli P, Martin J, Delmas L, Prieur B, Burgel PR | 2021-11-01 | Advanced CF disease, CFTR modulator, Grounded theory, Patient perspectives, Patient-reported outcomes, Quality of life | Respiratory medicine and research |
| Involvement of CFTR in the pathogenesis of pulmonary arterial hypertension. | Le Ribeuz H, To L, Ghigna MR, Martin C, Nagaraj C, Dreano E, Rucker-Martin C, Girerd B, Bouligand J, Pechoux C, Lambert M, Boet A, Issard J, Mercier O, Hoetzenecker K, Manoury B, Becq F, Burgel PR, Cottart CH, Olschewski A, Sermet-Gaudelus I, Perros F, Humbert M, Montani D, Antigny F | 2021-11-01 | | The European respiratory journal |
| [se-atlas.de-Medical care atlas for people with rare diseases]. | Neff M, Schaaf J, Tegtbauer N, Schäfer J, Till M, Wagner TOF, Graeßner H, Mundlos C, Storf H | 2021-10-01 | Care centers, Health facilities, Health personnel, Patient portals, Rare diseases | Der Internist |
| Tuberous sclerosis complex for the pulmonologist. | Rebaine Y, Nasser M, Girerd B, Leroux C, Cottin V | 2021-09-30 | | European respiratory review : an official journal of the European Respiratory Society |
| Recent developments in the treatment of small cell lung cancer. | Hiddinga BI, Raskin J, Janssens A, Pauwels P, Van Meerbeeck JP | 2021-09-30 | | European respiratory review : an official journal of the European Respiratory Society |
| Improved survival albeit with persistent disparities in prognosis for people with cystic fibrosis in European countries. | Martin C, Burgel PR | 2021-09-01 | | The European respiratory journal |
| Looking forward: key initiatives to improve the care of rare diseases and streamline the delivery of medicines and vaccines in Europe. | Humbert M, Simonds AK | 2021-09-01 | ERS, European Respiratory Society, WLD2021, World Lung Day, pulmonary medicine | American journal of physiology. Lung cellular and molecular physiology |
| Updated guidance on the management of children with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID). | Barben J, Castellani C, Munck A, Davies JC, de Winter-de Groot KM, Gartner S, Kashirskaya N, Linnane B, Mayell SJ, McColley S, Ooi CY, Proesmans M, Ren CL, Salinas D, Sands D, Sermet-Gaudelus I, Sommerburg O, Southern KW | 2021-09-01 | CFSPID, CRMS, Cystic fibrosis, Management, Newborn screening | Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society |
| Misconceptions regarding symptoms of sarcoidosis. | Drent M, Costabel U, Crouser ED, Grunewald J, Bonella F | 2021-08-01 | | The Lancet. Respiratory medicine |
| Reply to Kuek et al.: Optimism with Caution: Elexacaftor-Tezacaftor-Ivacaftor in Patients with Advanced Pulmonary Disease. | Burgel PR, Da Silva J, Paillasseur JL, Martin C | 2021-08-01 | | American journal of respiratory and critical care medicine |
| Radiological differences between chronic thromboembolic pulmonary disease (CTEPD) and chronic thromboembolic pulmonary hypertension (CTEPH). | Capone C, Valentini A, Spinillo SL, Klersy C, Celentano A, Pin M, Monterosso C, Dore R, Bassi EM, Zacchino M, Rodolico G, Corsico AG, Preda L, Ghio S, D'Armini AM | 2021-08-01 | Dual-energy computed tomography, Pulmonary embolism, Pulmonary hypertension | European radiology |
| Low income and outcome in idiopathic pulmonary fibrosis: An association to uncover. | Sesé L, Caliez J, Annesi-Maesano I, Cottin V, Pesce G, Didier M, Carton Z, Israel-Biet D, Crestani B, Dudoret SG, Cadranel J, Wallaert B, Tazi A, Maître B, Prévot G, Marchand-Adam S, Hirschi S, Dury S, Giraud V, Gondouin A, Bonniaud P, Traclet J, Juvin K, Borie R, Bernaudin JF, Valeyre D, Cavalin C, Nunes H | 2021-07-01 | Air pollution, Idiopathic pulmonary fibrosis, Income, Occupational exposure, Socioeconomic status | Respiratory medicine |
| Care Delivery Models and Interstitial Lung Disease: The Role of the Specialized Center. | Fisher JH, Cottin V | 2021-06-01 | Interstitial lung disease, Multidisciplinary team, Specialized center | Clinics in chest medicine |
| The role of cardiopulmonary exercise test in identifying pulmonary veno-occlusive disease. | Pérez-Olivares C, Segura de la Cal T, Flox-Camacho Á, Nuche J, Tenorio J, Martínez Meñaca A, Cruz-Utrilla A, de la Cruz-Bertolo J, Pérez Núñez M, Consortium SP, Arribas-Ynsaurriaga F, Escribano Subías P | 2021-06-01 | | The European respiratory journal |
| Mental Well-being and General Health in Adolescents with Asthma: The Prevention and Incidence of Asthma and Mite Allergy Birth Cohort Study. | van der Laan SEI, de Hoog MLA, Nijhof SL, Gehring U, Vonk JM, van der Ent CK, Wijga AH | 2021-06-01 | | The Journal of pediatrics |
| Estimates of epidemiology, mortality and disease burden associated with progressive fibrosing interstitial lung disease in France (the PROGRESS study). | Nasser M, Larrieu S, Boussel L, Si-Mohamed S, Bazin F, Marque S, Massol J, Thivolet-Bejui F, Chalabreysse L, Maucort-Boulch D, Hachulla E, Jouneau S, Le Lay K, Cottin V | 2021-05-24 | Algorithms, Epidemiology, Healthcare resource utilisation, Interstitial lung disease, Progressive fibrosis | Respiratory research |
| PH CARE COVID survey: an international patient survey on the care for pulmonary hypertension patients during the early phase of the COVID-19 pandemic. | Godinas L, Iyer K, Meszaros G, Quarck R, Escribano-Subias P, Vonk Noordegraaf A, Jansa P, D'Alto M, Luknar M, Milutinov Ilic S, Belge C, Sitbon O, Reis A, Rosenkranz S, Pepke-Zaba J, Humbert M, Delcroix M | 2021-05-01 | COVID-19, Chronic thromboembolic pulmonary hypertension, Patient survey, Pulmonary arterial hypertension, Pulmonary hypertension | Orphanet journal of rare diseases |
| Expectations about treatment of idiopathic pulmonary fibrosis: Comparative survey of patients, carers and physicians (the RESPIR French survey). | Cottin V, Bergot E, Bourdin A, Nunes H, Prévot G, Wallaert B, Marchand-Adam S | 2021-05-01 | Drug therapy, Idiopathic pulmonary fibrosis, Management, Patient and carer experience, Survey | Respiratory medicine and research |
| Educational aspects of rare and orphan lung diseases. | Alfaro TM, Wijsenbeek MS, Powell P, Stolz D, Hurst JR, Kreuter M, Moor CC | 2021-03-24 | Delayed diagnosis, Medical education, Medical societies, Patient education, Patient participation, Rare diseases | Respiratory research |
| Safety of G-CSF with concurrent chemo-radiotherapy in limited-stage small cell lung cancer - Secondary analysis of the randomised phase 3 CONVERT trial. | Gomes F, Faivre-Finn C, Mistry H, Bezjak A, Pourel N, Fournel P, Van Meerbeeck J, Blackhall F | 2021-03-01 | Concurrent chemo-radiotherapy, G-CSF, SCLC, Small cell lung cancer, cCTRT | Lung cancer (Amsterdam, Netherlands) |
| Clinical response to lumacaftor-ivacaftor in patients with cystic fibrosis according to baseline lung function. | Burgel PR, Durieu I, Chiron R, Mely L, Prevotat A, Murris-Espin M, Porzio M, Abely M, Reix P, Marguet C, Macey J, Sermet-Gaudelus I, Corvol H, Bui S, Biouhee T, Hubert D, Munck A, Lemonnier L, Dehillotte C, Silva JD, Paillasseur JL, Martin C | 2021-03-01 | | Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society |
| Cystic Fibrosis Newborn Screening in Austria Using PAP and the Numeric Product of PAP and IRT Concentrations as Second-Tier Parameters. | Zeyda M, Schanzer A, Basek P, Bauer V, Eber E, Ellemunter H, Kallinger M, Riedler J, Thir C, Wadlegger F, Zacharasiewicz A, Renner S | 2021-02-13 | IRT-PAP, IRT×PAP, false-positives, neonatal screening, recalls | Diagnostics (Basel, Switzerland) |
| Hemodynamic Response to Treatment and Outcomes in Pulmonary Hypertension Associated With Interstitial Lung Disease Versus Pulmonary Arterial Hypertension in Systemic Sclerosis: Data From a Study Identifying Prognostic Factors in Pulmonary Hypertension Associated With Interstitial Lung Disease. | Chauvelot L, Gamondes D, Berthiller J, Nieves A, Renard S, Catella-Chatron J, Ahmad K, Bertoletti L, Camara B, Gomez E, Launay D, Montani D, Mornex JF, Prévot G, Sanchez O, Schott AM, Subtil F, Traclet J, Turquier S, Zeghmar S, Habib G, Reynaud-Gaubert M, Humbert M, Cottin V | 2021-02-01 | | Arthritis & rheumatology (Hoboken, N.J.) |
| Radiological Findings in Multidetector Computed Tomography (MDCT) of Hereditary and Sporadic Pulmonary Veno-Occlusive Disease: Certainties and Uncertainties. | Pérez Núñez M, Alonso Charterina S, Pérez-Olivares C, Revilla Ostolaza Y, Morales Ruiz R, Enguita Valls AB, Tenorio JA, Gallego Zazo N, De Pablo Gafas A, Lapunzina P, Rodríguez Chaverri A, Escribano Subías P | 2021-01-19 | multidetector computed tomography (MDCT), pulmonary hypertension, pulmonary veno-occlusive disease | Diagnostics (Basel, Switzerland) |
| Mortality and Respiratory-Related Hospitalizations in Idiopathic Pulmonary Fibrosis Not Treated With Antifibrotics. | Cottin V, Spagnolo P, Bonniaud P, Nolin M, Dalon F, Kirchgässler KU, Kamath TV, Van Ganse E, Belhassen M | 2021-01-01 | acute hospitalizations, antifibrotics, claims data, idiopathic pulmonary fibrosis, mortality | Frontiers in medicine |
| Variability in Global Prevalence of Interstitial Lung Disease. | Kaul B, Cottin V, Collard HR, Valenzuela C | 2021-01-01 | epidemiology—descriptive, global epidemiology, idiopathic pulmonary fibrosis, interstitial lung disease, mortality | Frontiers in medicine |
| Epidemiology, Mortality and Healthcare Resource Utilization Associated With Systemic Sclerosis-Associated Interstitial Lung Disease in France. | Cottin V, Larrieu S, Boussel L, Si-Mohamed S, Bazin F, Marque S, Massol J, Thivolet-Bejui F, Chalabreysse L, Maucort-Boulch D, Jouneau S, Hachulla E, Chollet J, Nasser M | 2021-01-01 | cost, epidemiology, pulmonary fibrosis, scleroderma, systemic sclerosis | Frontiers in medicine |
| Gender Differences in Idiopathic Pulmonary Fibrosis: Are Men and Women Equal? | Sesé L, Nunes H, Cottin V, Israel-Biet D, Crestani B, Guillot-Dudoret S, Cadranel J, Wallaert B, Tazi A, Maître B, Prévot G, Marchand-Adam S, Hirschi S, Dury S, Giraud V, Gondouin A, Bonniaud P, Traclet J, Juvin K, Borie R, Carton Z, Freynet O, Gille T, Planès C, Valeyre D, Uzunhan Y | 2021-01-01 | gender differences, idiopathic pulmonary fibrosis, lung transplantation, occupational exposures, women | Frontiers in medicine |
| The Experience, Prerequisites, and the Barriers in Organizing a Specialized Rehabilitation Program for Patients with Pulmonary Hypertension. | Palevičiūtė E, Gumbienė L, Jurevičienė E, Šimbelytė T, Laucevičienė I, Laucevičius A, Barysienė J, Eichstaedt CA, Benjamin N, Grünig E, Čelutkienė J | 2021-01-01 | Exercise program, Implementation, Pulmonary hypertension, Rehabilitation, Training | Respiration; international review of thoracic diseases |
| Concurrence of 1- and 3-Min Sit-to-Stand Tests with the 6-Min Walk Test in Idiopathic Pulmonary Fibrosis. | Fedi A, Keddache S, Quétant S, Guillien A, Antoniadis A, Soumagne T, Ritter O, Glérant JC, Cottin V, Degano B, Aguilaniu B | 2021-01-01 | 6-min walk test, Idiopathic pulmonary fibrosis, Sit-to-stand tests | Respiration; international review of thoracic diseases |
| Update on Respiratory Fungal Infections in Cystic Fibrosis Lung Disease and after Lung Transplantation. | Renner S, Nachbaur E, Jaksch P, Dehlink E | 2020-12-21 | ABPA, antifungals, fungi, respiratory exacerbation | Journal of fungi (Basel, Switzerland) |
| Quantification of Cystic Fibrosis Lung Disease with Radiomics-based CT Scores. | Chassagnon G, Zacharaki EI, Bommart S, Burgel PR, Chiron R, Dangeard S, Paragios N, Martin C, Revel MP | 2020-12-01 | | Radiology. Cardiothoracic imaging |
| Reduced Intestinal Inflammation With Lumacaftor/Ivacaftor in Adolescents With Cystic Fibrosis. | Tétard C, Mittaine M, Bui S, Beaufils F, Maumus P, Fayon M, Burgel PR, Lamireau T, Delhaes L, Mas E, Enaud R | 2020-12-01 | | Journal of pediatric gastroenterology and nutrition |
| Feasibility of a Noninvasive Operability Assessment in Chronic Thromboembolic Pulmonary Hypertension under Real-World Practice. | Rodriguez Chaverri A, Revilla Ostolaza Y, Lopez-Gude MJ, Velazquez MT, Ponz de Antonio I, Alonso Charterina S, Albarran Gonzalez-Trevilla A, Perez Nunez M, Perez Vela JL, Morales Ruiz R, Delgado Jimenez JF, Arribas Ynsaurriaga F, Cortina JM, Escribano Subias P | 2020-10-21 | endarterectomy, hypertension, pulmonary, pulmonary embolism | Diagnostics (Basel, Switzerland) |
| Urinary Exosomes of Patients with Cystic Fibrosis Unravel CFTR-Related Renal Disease. | Gauthier S, Pranke I, Jung V, Martignetti L, Stoven V, Nguyen-Khoa T, Semeraro M, Hinzpeter A, Edelman A, Guerrera IC, Sermet-Gaudelus I | 2020-09-10 | cystic fibrosis, cystic fibrosis transmembrane conductance regulator, kidney, klotho, proteomic | International journal of molecular sciences |
| Current treatment of sarcoidosis. | Sellarés J, Francesqui J, Llabres M, Hernandez-Gonzalez F, Baughman RP | 2020-09-01 | | Current opinion in pulmonary medicine |
| R117H-CFTR function and response to VX-770 correlate with mRNA and protein expression in intestinal organoids. | Van Mourik P, van Haaren P, Kruisselbrink E, Korkmaz C, Janssens HM, de Winter-de Groot KM, van der Ent CK, Hagemeijer MC, Beekman JM | 2020-09-01 | CFTR, Organoids, R117H, VX-770, mRNA | Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society |
| Prediction of Prognostic Hemodynamic Indices in Pulmonary Hypertension Using Non-Invasive Parameters. | Mańczak R, Kurzyna M, Piłka M, Darocha S, Florczyk M, Wieteska-Miłek M, Mańczak M, Torbicki A | 2020-08-27 | biomarkers, echocardiography, logistic regression, prognostic indices, pulmonary hypertension, right heart catheterization, risk stratification | Diagnostics (Basel, Switzerland) |
| Desquamative interstitial pneumonia: a systematic review of its features and outcomes. | Hellemons ME, Moor CC, von der Thüsen J, Rossius M, Odink A, Thorgersen LH, Verschakelen J, Wuyts W, Wijsenbeek MS, Bendstrup E | 2020-06-30 | | European respiratory review : an official journal of the European Respiratory Society |
| Diagnostic approach of fibrosing interstitial lung diseases of unknown origin. | Cottin V, Valenzuela C | 2020-06-01 | | Presse medicale (Paris, France : 1983) |
| Patients' and Healthcare Professionals' Experiences of Idiopathic Pulmonary Fibrosis Treatment with the Pirfenidone 801 mg Tablet Formulation: A Multinational Survey. | Lancaster LH, Valenzuela C, Mason W, Neurohr C, Ripamonti E, Kirchgaessler KU, Cottin V | 2020-06-01 | Antifibrotics, Idiopathic pulmonary fibrosis, Pill burden, Pirfenidone, Quality of life, Questionnaire | Pulmonary therapy |
| Impact of COVID-19 on people with cystic fibrosis. | Colombo C, Burgel PR, Gartner S, van Koningsbruggen-Rietschel S, Naehrlich L, Sermet-Gaudelus I, Southern KW | 2020-05-01 | | The Lancet. Respiratory medicine |
| Antimicrobial resistance in cystic fibrosis: A Delphi approach to defining best practices. | Zemanick E, Burgel PR, Taccetti G, Holmes A, Ratjen F, Byrnes CA, Waters VJ, Bell SC, VanDevanter DR, Stuart Elborn J, Flume PA | 2020-05-01 | Antimicrobial resistance, Consensus, Cystic fibrosis | Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society |
| Getting neural about airway gland secretion. | Amatngalim GD, Ribeiro CMP | 2020-04-01 | | The European respiratory journal |
| Prolidase deficiency: a new genetic cause of combined pulmonary fibrosis and emphysema syndrome in the adult. | Cottin V, Nasser M, Traclet J, Chalabreysse L, Lèbre AS, Si-Mohamed S, Philit F, Thivolet-Béjui F | 2020-04-01 | | The European respiratory journal |
| CheckMate 171: A phase 2 trial of nivolumab in patients with previously treated advanced squamous non-small cell lung cancer, including ECOG PS 2 and elderly populations. | Felip E, Ardizzoni A, Ciuleanu T, Cobo M, Laktionov K, Szilasi M, Califano R, Carcereny E, Griffiths R, Paz-Ares L, Duchnowska R, Garcia MA, Isla D, Jassem J, Appel W, Milanowski J, Van Meerbeeck JP, Wolf J, Li A, Acevedo A, Popat S | 2020-03-01 | Comorbidity, Elderly, Health status indicators, Nivolumab, Non-small cell lung cancer | European journal of cancer (Oxford, England : 1990) |
| Genetic diagnosis in practice: From cystic fibrosis to CFTR-related disorders. | Pagin A, Sermet-Gaudelus I, Burgel PR | 2020-02-01 | CFTR-related disorders (CFTR-RD), CFTR-related metabolic syndrome(CRMS)/CF screen positive inconclusive diagnosis (CFSPID), Cystic fibrosis (CF), Cystic fibrosis transmembrane conductance regulator (CFTR) | Archives de pediatrie : organe officiel de la Societe francaise de pediatrie |
| Real-Life Safety and Effectiveness of Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis. | Burgel PR, Munck A, Durieu I, Chiron R, Mely L, Prevotat A, Murris-Espin M, Porzio M, Abely M, Reix P, Marguet C, Macey J, Sermet-Gaudelus I, Corvol H, Bui S, Lemonnier L, Dehillotte C, Da Silva J, Paillasseur JL, Hubert D | 2020-01-15 | cystic fibrosis, lumacaftor–ivacaftor, postmarketing study | American journal of respiratory and critical care medicine |
| Risk and outcome of COVID-19 infection in sarcoidosis patients: results of a self-reporting questionnaire. | Baughman RP, Lower EE, Buchanan M, Rottoli P, Drent M, Sellares J, Terwiel M, Elfferich M, Francesqui J, Barriuso Cabrerizo MR, Sweiss N, Martone F, Al-Hakim T, Judson MA | 2020-01-01 | COVID-19, immunosuppression, sarcoidosis | Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG |
| Validation of short- and long-term demographic forecasts using the Canadian Cystic Fibrosis Registry. | Martelli V, Sykes J, Burgel PR, Bellis G, Coriati A, Stanojevic S, Stephenson AL | 2020-01-01 | | The European respiratory journal |
| Cystic Fibrosis Diagnosis in Newborns, Children, and Adults. | Castellani C, Linnane B, Pranke I, Cresta F, Sermet-Gaudelus I, Peckham D | 2019-12-01 | | Seminars in respiratory and critical care medicine |
| Tumour Treating Fields in combination with pemetrexed and cisplatin or carboplatin as first-line treatment for unresectable malignant pleural mesothelioma (STELLAR): a multicentre, single-arm phase 2 trial. | Ceresoli GL, Aerts JG, Dziadziuszko R, Ramlau R, Cedres S, van Meerbeeck JP, Mencoboni M, Planchard D, Chella A, Crinò L, Krzakowski M, Rüssel J, Maconi A, Gianoncelli L, Grosso F | 2019-12-01 | | The Lancet. Oncology |
| Association between surgical volume and post-operative mortality and survival after surgical resection in lung cancer in Belgium: A population-based study. | Schillemans V, Vrijens F, De Gendt C, Robays J, Silversmit G, Verleye L, Camberlin C, Dubois C, Stordeur S, Wauters I, Van Meerbeeck JP, Van Eycken E, De Leyn P | 2019-12-01 | Hospital surgical volume, Lung cancer, Mortality, Survival | European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology |
| Influence of Antisynthetase Antibodies Specificities on Antisynthetase Syndrome Clinical Spectrum Time Course. | Cavagna L, Trallero-Araguás E, Meloni F, Cavazzana I, Rojas-Serrano J, Feist E, Zanframundo G, Morandi V, Meyer A, Pereira da Silva JA, Matos Costa CJ, Molberg O, Andersson H, Codullo V, Mosca M, Barsotti S, Neri R, Scirè C, Govoni M, Furini F, Lopez-Longo FJ, Martinez-Barrio J, Schneider U, Lorenz HM, Doria A, Ghirardello A, Ortego-Centeno N, Confalonieri M, Tomietto P, Pipitone N, Rodriguez Cambron AB, Blázquez Cañamero MÁ, Voll RE, Wendel S, Scarpato S, Maurier F, Limonta M, Colombelli P, Giannini M, Geny B, Arrigoni E, Bravi E, Migliorini P, Mathieu A, Piga M, Drott U, Delbrueck C, Bauhammer J, Cagnotto G, Vancheri C, Sambataro G, De Langhe E, Sainaghi PP, Monti C, Gigli Berzolari F, Romano M, Bonella F, Specker C, Schwarting A, Villa Blanco I, Selmi C, Ceribelli A, Nuno L, Mera-Varela A, Perez Gomez N, Fusaro E, Parisi S, Sinigaglia L, Del Papa N, Benucci M, Cimmino MA, Riccieri V, Conti F, Sebastiani GD, Iuliano A, Emmi G, Cammelli D, Sebastiani M, Manfredi A, Bachiller-Corral J, Sifuentes Giraldo WA, Paolazzi G, Saketkoo LA, Giorgi R, Salaffi F, Cifrian J, Caporali R, Locatelli F, Marchioni E, Pesci A, Dei G, Pozzi MR, Claudia L, Distler J, Knitza J, Schett G, Iannone F, Fornaro M, Franceschini F, Quartuccio L, Gerli R, Bartoloni E, Bellando Randone S, Zampogna G, Gonzalez Perez MI, Mejia M, Vicente E, Triantafyllias K, Lopez-Mejias R, Matucci-Cerinic M, Selva-O'Callaghan A, Castañeda S, Montecucco C, Gonzalez-Gay MA | 2019-11-18 | antisynthetase antibodies, antisynthetase syndrome, arthritis, interstitial lung disease, myositis | Journal of clinical medicine |
| Precision medicine: The future of diagnostic approach to pulmonary hypertension? | Kedzierski P, Torbicki A | 2019-09-01 | | Anatolian journal of cardiology |
| Where are the gaps in education in the field of rare lung disease? Perspectives from the ERN-LUNG educational programme survey. | Powell P, Kreuter M, Wijsenbeek-Lourens M | 2019-06-01 | | Breathe (Sheffield, England) |
| The Ariane-IPF ERS Clinical Research Collaboration: seeking collaboration through launch of a federation of European registries on idiopathic pulmonary fibrosis. | Cottin V, Annesi-Maesano I, Günther A, Galvin L, Kreuter M, Powell P, Prasse A, Reynolds G, Richeldi L, Spagnolo P, Valenzuela C, Wijsenbeek M, Wuyts WA, Crestani B | 2019-05-01 | | The European respiratory journal |
