Vasculitis and granulomatosis in the respiratory tract

Lately new aspects of diseases have been described in long known forms of pulmonary vasculitis and granulomatosis like Wegener's granulomatosis (WG), sarcoidosis (SA), exogenous allergic alveolitis and the Churg-Strauss syndrome. In WG it is clinically important to distinguish between two different phases of disease - the early initial phase with localized inflammation and granuloma formation and the later generalized form of disease where an involvement of virtually every organ system can occur. Recently autoantibodies (antineutrophil cytoplasmic antibodies) have been discovered which are highly specific for WG and additionally are helpful to determine the activity of the disease. With respect to both findings and more recent experience with various therapeutic regimes a therapy considering the phase and activity of disease is recommended. In SA the analysis of the bronchoalveolar lavage has not only led to a immunodiagnostic concept but also given immunopathogenic insights. In contrast to former ideas the SA is not the consequence of an anergic state but more likely of an increased activity of the immune system which is hinted at by the finding of activated CD4-positive lymphocytes in the interstitial tissue and lavage fluid. In exogenous allergic alveolitis new disease concepts are arising from the better understanding of the triggering agents which can now be better analysed.