Pathologie pulmonaler Beteiligung bei Vaskulitiden und Systemerkrankungen

Manifestations of pulmonary vasculitis syndromes and collagen vascular diseases are extremely variable and diagnostically challenging. Pulmonary involvement is frequent in the primary systemic vasculitis (PSV) syndromes Wegener's granulomatosis, Churg-Strauss syndrome and microscopic polyangiitis. PSV have to be discriminated from pulmonary involvement in other rheumatic diseases, secondary vasculitis due to infections and vascular affections secondary to lymphoproliferative diseases. Pulmonary involvement occurs in all types of collagen vascular diseases, and morphology may be identical in different types of rheumatic diseases as well as idiopathic interstitial lung disease. The diagnostic challenge here is to discriminate collagen vascular disease from idiopathic lung disease, from adverse drug effects and infections. In many cases, not all diagnostic criteria can be found in a single biopsy; therefore, the selection of appropriate biopsy material as well as a correlation with clinical, serological and radiological parameters is essential.