The world of rare interstitial lung diseases

被引：7

作者：

Buschulte, Katharina ^{[1
]}

Cottin, Vincent ^{[2
]}

Wijsenbeek, Marlies ^{[3
]}

Kreuter, Michael ^{[1
]}

Diesler, Remi ^{[2
]}

机构：

[1] Heidelberg Univ, Ctr Interstitial & Rare Lung Dis, German Ctr Lung Res DZL, Thoraxklin,ERN LUNG, Heidelberg, Germany

[2] Claude Bernard Univ Lyon 1, Louis Pradel Hosp, Natl Reference Ctr Rare Pulm Dis, Hosp Civils Lyon,UMR 754,ERN LUNG, Lyon, France

[3] Erasmus MC Univ Med Ctr, Ctr Interstitial Lung Dis & Sarcoidosis, Dept Resp Med, ERN LUNG, Rotterdam, Netherlands

来源：

EUROPEAN RESPIRATORY REVIEW | 2023年 / 32卷 / 167期

关键词：

PULMONARY ALVEOLAR MICROLITHIASIS; HERMANSKY-PUDLAK-SYNDROME; LANGERHANS-CELL HISTIOCYTOSIS; IDIOPATHIC PLEUROPARENCHYMAL FIBROELASTOSIS; SURFACTANT PROTEIN-C; HOGG-DUBE-SYNDROME; CLINICAL-FEATURES; BRONCHOALVEOLAR LAVAGE; PATHOLOGICAL FEATURES; RITUXIMAB THERAPY;

D O I：

10.1183/16000617.0161-2022

中图分类号：

R56 [呼吸系及胸部疾病];

学科分类号：

摘要：

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.

引用

页数：19

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