Trimethoprim-Sulfamethoxazole for Scleritis Associated with Limited Wegenerʼs Granulomatosis: Use of Histopathology and Anti-neutrophil Cytoplasmic Antibody (ANCA) Test

Ophthalmic involvement may be noted in ≤ 58% of Wegenerʼs granulomatosis cases, scleritis being one of the most frequent and potentially devastating manifestations. Cytotoxic immunosuppressive drug therapy is effective treatment for this disorder but potentially highly toxic. Recent uncontrolled and anecdotal reports have suggested a possible therapeutic role for a much less toxic agent, trimethoprim/sulfamethoxazole, in limited Wegenerʼs granulomatosis. We report a patient who had a conjunctival nodule and scleritis. Biopsy of the nodule suggested Wegenerʼs granulomatosis, confirmed serologically with serum anti-neutrophil cytoplasmic antibody (ANCA) testing. Treatment with oral trimethoprim/ sulfamethoxazole was successful. Clinical response was paralleled by normalization of serial anti-neutrophil cytoplasmic antibody titers. This case is the first well-documented ophthalmologic report of limited Wegenerʼs granulomatosis responding to trimethoprim/sulfamethoxazole and adds to the body of literature suggesting a potential role for this drug in selected cases of limited Wegenerʼs granulomatosis.