Clinical features of primary vasculitides of the ENT region

Strauss syndrome (CSS) and microscopic polyangiitis (MPA), frequently affec t the ENT region. For several reasons WG is of special significance for the otorhinolaryngologist. First, disease activity limited to the upper respir atory tract (localized WG) often proceeds the systemic vasculitis (generali zed WG). The early diagnosis therefore has decisive consequences for stage adapted therapy. Second, in most cases (nearly 80%) WG is diagnosed histolo gically on biopsy specimens from the ENT region. During the initial phase o f WG this is of diagnostic relevance, because at this stage the serologic p arameters (acute-phase proteins) usually have a normal value and PR3-ANCA i s (still) negative in 2/3 of the patients. Third, in many cases recurrences reveal increased activity in the ENT region, or start in this area. Clinic ally in most cases chronic rhinosinusitis with crusting and epistaxis is se en, sometimes with septal perforation and/or saddle nose. Apart from this t here are often unclear middle ear symptoms with recurrent effusions and the inner ear is sometimes also affected. Laryngeal manifestations are typical ly located in the subglottic area and lead to subglottic stenosis. In the d ifferential diagnosis, diseases in which epitheloid cell granulomas occur, such as sarcoidosis and TBC, need to be considered, but also foreign body g ranulomas and fungus diseases. Finally malignant tumors, especially maligna nt lymphomas, have to be ruled out.