ABSTRACT
Primary systemic vasculitic diseases are relatively rare. Untreated, they have a high morbidity and mortality. The introduction of steroids and cytotoxic drugs has dramatically reduced the mortality. In a retrospective study in a Norwegian community hospital, which serves a county with a population of 150,426 in 1996, 68 patients were found to have a primary vasculitis, 63 of whom fulfilled the ACR 1990 criteria. Patients with Henoch-Schönlein purpura aged less than 16 years and patients with Kawasaki's disease were excluded. The overall prevalence was 43.9 per 100,000 inhabitants (Churg-Strauss syndrome 1.3, hypersensitivity vasculitis 2.7, Henoch-Schönlein purpura 3.3, polyarteritis nodosa 3.3, Wegener's granulomatosis 5.3 and temporal arteritis 27.9). In most cases, disease control was achieved with corticosteroids alone, or with the addition of cytotoxic drugs. Two patients had died in the latest 5-year period but of unrelated disorders. Biopsy plays a major role in diagnosing vasculitic diseases. In our study, 62 patients had a positive biopsy supporting the diagnosis.
