Purpose: To evaluate possible factors associated with disease relapse
in patients with ocular manifestations of Wegener granulomatosis. Meth
ods: Eight patients with ocular manifestations of Wegener granulomatos
is 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 o
r scleritis combined with peripheral ulcerative keratitis. Clinical di
sease remission was achieved in all patients using immunosuppressive c
hemotherapy. Five patients had subsequent relapse after treatment with
drawal. The serum ANCA level had failed to revert to normal during rem
ission in four of the five patients who had had relapses. The ANCA lev
els for all three patients who remained in remission without therapy c
onverted to normal. There was no significant difference in the initial
serum ANCA level (P = 0.35) or the mean cumulative cyclophosphamide d
ose (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 a
ssociated with the potential for relapse in patients with ocular manif
estations of Wegener granulomatosis.