Lungenbeteiligung bei systemischen vaskulitiden

Systemic vasculitides that affect the lung parenchyma are mainly small-vessel vasculitides. Owing to its dual character with granulomatous disease of the respiratory tract on the one hand and pulmonary and extrapulmonary vasculitis on the other, the clinical spectrum of Wegener's granulomatosis is highly variable. Much has been learned about the immunopathogenesis of its granulomatous facet. Likewise, the insight into the pathogenesis of vasculitic disease has been refined. The propensity of WG for lung disease may result from the presence of proteinase 3, which is the target antigen of PR3-ANCA, not only in myeloic but also non-myeloic cells, e.g. in types I and II pneumocytes. The pathogenetic understanding of the pulmonary manifestations of microscopic polyangiitis is still incomplete and this applies particularly to the fibrosing alveolitis in this disease. The relationships between Churg Strauss syndrome (CSS) and certain immunomodulators used in asthma treatment have been examined in numerous patients. A direct causal relation between agents like the leukotriene inhibitors and the initiation of CSS appears to be unlikely.