TY - JOUR
T1 - The world of rare interstitial lung diseases
AU - Buschulte, Katharina
AU - Cottin, Vincent
AU - Wijsenbeek, Marlies
AU - Kreuter, Michael
AU - Diesler, Rémi
N1 - Publisher Copyright:
© The authors 2023.
PY - 2023/3/1
Y1 - 2023/3/1
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85147723775&partnerID=8YFLogxK
U2 - 10.1183/16000617.0161-2022
DO - 10.1183/16000617.0161-2022
M3 - Article
C2 - 36754433
AN - SCOPUS:85147723775
SN - 0905-9180
VL - 32
JO - European Respiratory Review
JF - European Respiratory Review
IS - 167
M1 - 220161
ER -