CLINICAL-FEATURES AND OUTCOME IN PATIENTS WITH GLOMERULONEPHRITIS ANDANTINEUTROPHIL CYTOPLASMIC AUTOANTIBODIES

We investigated the clinical features and outcome of 60 patients with antineutrophil cytoplasmic autoantibodies with cytoplamic staining (C- ANCA)- and with (peri)nuclear staining (P-ANCA)-associated glomerulone phritis. Virtually all patients with C-ANCA had antibodies against pro teinase 3, which corresponded with a clinical and/or histological diag nosis of Wegener's granulomatosis (WG). P-ANCA were associated with an tibodies against myeloperoxidase. Although more often associated with renal-limited disease, a significant number of patients with P-ANCA ha d also symptoms of organs known to be preferentially affected with WG. The majority of patients presented with rapidly progressive glomerulo nephritis which responded favorably to immunosuppressive therapy, albe it at the expense of a considerable number of fatal infections. Furthe rmore, a more protracted form of glomerulonephritis was recognized. A kidney biopsy scoring system appeared to be of value in predicting whi ch patients would benefit most from treatment. C-ANCA and P-ANCA appea r to identify a subset of patients with vasculitis that share common c linical features and a favorable response to immunosuppressive therapy .