Disease relapse in patients with ocular manifestations of Wegener granulomatosis.

PURPOSE: To evaluate possible factors associated with disease relapse in patients with ocular manifestations of Wegener granulomatosis.

METHODS

Eight patients with ocular manifestations of Wegener granulomatosis were longitudinally followed for a mean period of 34 months. Serial antineutrophil cytoplasmic antibody (ANCA) levels were determined on all patients.

RESULTS

All eight patients had either scleritis alone or scleritis combined with peripheral ulcerative keratitis. Clinical disease remission was achieved in all patients using immunosuppressive chemotherapy. Five patients had subsequent relapse after treatment withdrawal. The serum ANCA level had failed to revert to normal during remission in four of the five patients who had had relapses. The ANCA levels for all three patients who remained in remission without therapy converted to normal. There was no significant difference in the initial serum ANCA level (P = 0.35) or the mean cumulative cyclophosphamide dose (P = 0.13) between those who had a relapse and those who did not.

CONCLUSION

Failure of ANCA titers to revert to normal levels may be associated with the potential for relapse in patients with ocular manifestations of Wegener granulomatosis.