Serial measurements of antineutrophil cytoplasmic autoantibodies in patients with systemic vasculitis

PURPOSE: To assess the value of serial determinations of antineutrophil cyt oplasmic autoantibodies (ANCA) for monitoring disease activity in patients with systemic vasculitis. PATIENTS AND METHODS: Forty-three patients with histologically proven vascu litis (21 with Wegener's granulomatosis, 17 with microscopic polyangiitis, and 5 with renal-limited vasculitis) were studied for a median follow-up of 22 months. Disease activity was prospectively assessed and quantified by t he Birmingham Vasculitis Activity Score. A total of 347 sera were analyzed for ANCA determination. RESULTS: Relapses occurred in 23 (54%) of 43 patients. Diagnostic category (Wegener's granulomatosis vs micropolyangiitis and renal-limited vasculitis ), severity of initial symptoms (mean vasculitis activity score, mean numbe r of organs involved), and ANCA pattern [cytoplasmic-ANCA (c-ANCA) vs perin uclear-ANCA (p-ANCA)] did not significantly differ between relapsers and no nrelapsers. Lung involvement was more frequent at onset among relapsers [16 of 23 (70%) vs 6 of 20 (30%); P = 0.02]. Relapses were slightly, but not s ignificantly, more frequent in patients with Wegener's granulomatosis or a c-ANCA pattern. The percentage of relapsers was greater in patients with pe rsistently positive ANCA than in patients with negative or decreasing ANCA titers (86% vs 20%, P = 0.0001). However, the predictive value of an increa se in ANCA titers for the occurrence of a subsequent relapse was only 28% ( 4 of 14) for c-ANCA, 12% (2 of 17) for anti-proteinase 3-ANCA, and 43% (6 o f 14) for anti-myeloperoxidase-ANCA. An increase in ANCA occurred before or during relapse in 33% (10 of 30) of cases for c-ANCA/anti-proteinase 3 ant ibodies, and 73% (11 of 15) of cases for anti-myeloperoxidase antibodies. CONCLUSION: The persistence of ANCA positivity is strongly associated with relapses. However, an increase in ANCA titers has a poor value for the earl y prediction of a subsequent relapse and should not be used as a sole param eter for therapeutic intervention. In addition, our results suggest that se rial anti-myeloperoxidase determination may be useful as a prognostic marke r in patients who are p-ANCA positive. (C) 1999 by Excerpta Medica, Inc.