The world of rare interstitial lung diseases.

The world of rare interstitial lung diseases (ILDs) is diverse and complex. Diagnosis and therapy usually pose challenges. This review describes a selection of rare and ultrarare ILDs including pulmonary alveolar proteinosis, pulmonary alveolar microlithiasis and pleuroparenchymal fibroelastosis. In addition, monogenic ILDs or ILDs in congenital syndromes and various multiple cystic lung diseases will be discussed. All these conditions are part of the scope of the European Reference Network on rare respiratory diseases (ERN-LUNG). Epidemiology, pathogenesis, diagnostics and treatment of each disease are presented.

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TitleThe world of rare interstitial lung diseases.
Date2023-03-31
Issue nameEuropean respiratory review : an official journal of the European Respiratory Society
Issue numberv32.167
DOI10.1183/16000617.0161-2022
PubMed36754433
AuthorsBuschulte K, Cottin V, Wijsenbeek M, Kreuter M & Diesler R
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