The world of rare interstitial lung diseases

Katharina Buschulte, Vincent Cottin, Marlies Wijsenbeek, Michael Kreuter*, Rémi Diesler

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

4 Citations (Scopus)
27 Downloads (Pure)


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.

Original languageEnglish
Article number220161
JournalEuropean Respiratory Review
Issue number167
Publication statusPublished - 1 Mar 2023

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© The authors 2023.


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