Isolated abdominal vasculitis as an atypical presentation of Wegener's granulomatosis

Although current classifications characterize vasculitic syndromes based upon the size of the vessels involved, the histopathology, and the presence or absence of antineutrophil cytoplasmatic antibodies ANCA (1-3), those occasional patients with vasculitis whose features are not typical may evade diagnosis and effective treatment. We report one such patient who presented with bilateral refractory uveitis and abdominal angina who had a positive C-ANCA. Because of his atypical presentation, this patient's disease progressed over 8 yr despite an extensive gastrointestinal evaluation, before a diagnosis of vasculitis was established angiographically, and immunosuppressive therapy was begun.