Persistence of antineutrophil cytoplasmic antibodies (ANCA) in asymptomatic patients with systemic polyarteritis nodosa or Churg-Strauss syndrome: follow-up of 53 patients.

OBJECTIVE

We retrospectively analyzed the significance of persistent ANCA positivity after clinical remission in 53 consecutive patients with histologically and/or angiographically proven polyarteritis nodosa (PAN), or Churg-Strauss syndrome (CSS) followed between 1981 and 1993.

METHODS

ANCA were detected using an immunofluorescence assay and ELISA: Each patient met the American College of Rheumatology 1990 criteria for PAN or CSS. Clinical and biological evaluations were always essential factors in the decision to intensify therapy.

RESULTS

ANCA were initially present in 15 patients (28.3%): 3/26 (11.5%) with HBV-related PAN, 6/18 (33.3%) with PAN of unknown etiology and 6/9 (66.7%) with CSS. Five patients remained ANCA-positive after clinical remission: 3 with PAN (one of them relapsed) and 2 with CSS who both relapsed. Among the 12 patients who died during follow-up, only 1 (8.3%) was initially ANCA-positive. Fifteen of the 41 survivors (29.2%) were ANCA-positive.

CONCLUSION

Persistence of ANCA positivity in PAN and CSS may be a marker of an underlying disease process, but does not adequately reflect disease activity and, thus, in no case should be the only indication for therapeutic intensification.