Interstitielle Lungenerkrankung bei vaskulitiden und kollagenosen

Translated title of the contribution: Interstitial lung disease in vasculitides and collagen vascular diseases

A. Schnabel, W. L. Gross


Systemic vasculitides that affect the lung parenchyma are mainly small-vessel vasculitides. The clinical appearance of Wegener's granulomatosis, which is the most common disease in this category, is well characterized and much has been learned about the immunopathogenesis of its granulomatous and vasculitic manifestations. The spectrum of the pulmonary manifestations of microscopic polyangiitis is being recognized increasingly. Fibrosing alveolitis is a now well-perceived form of pulmonary involvement in this disease, although its pathogenesis and treatment requirements remain to be clarified. The syndrome of diffuse alveolar hemorrhage due to pulmonary capillaritis evolved to be an important and often life-threatening manifestation of small-vessel vasculitides. It requires immediate diagnosis and vigorous immunosuppressive treatment. Fibrosing alveolitis due to systemic sclerosis and other collagen vascular diseases is commonly less aggressive than idiopathic fibrosing alveolitis. It can take either a relentlessly progressive or a self-limiting disease course. Intense immunosuppression with daily oral or intravenous pulse cyclophosphamide has been found to halt disease progression, provided that it is instituted early on and targets inflammatory but not fibrous interstitial disease.

Translated title of the contributionInterstitial lung disease in vasculitides and collagen vascular diseases
Original languageGerman
JournalAtemwegs- und Lungenkrankheiten
Issue number11
Pages (from-to)532-546
Number of pages15
Publication statusPublished - 11.2003

Research Areas and Centers

  • Academic Focus: Center for Infection and Inflammation Research (ZIEL)


Dive into the research topics of 'Interstitial lung disease in vasculitides and collagen vascular diseases'. Together they form a unique fingerprint.

Cite this