Successful treatment of serpiginous choroiditis with alkylating agents

Objective: To describe the management and long-term outcomes of patients with serpiginous choroiditis treated with alkylating agents. Design: Retrospective, noncomparative case series. Participants: Nine patients with active, vision-threatening serpiginous choroiditis who had progressive inflammation while on steroids and/or immunosuppressive agents other than alkylating agents treated at three tertiary care uveitis referral centers. Methods: Patients received systemic immunosuppression with an alkylating agent, either chlorambucil or cyclophosphamide. Prednisone also was given initially and was tapered and discontinued. Main Outcome Measures: Visual acuity, clinical disease activity, duration of treatment, duration of drug-free disease remission, and side effects of alkylating agent therapy. Results: No patients had recurrences while on therapy. No further visual loss was encountered after starting the therapy. Six of the patients regained vision. All but two patients achieved prolonged drug-free remissions, ranging in duration between 15 and 96 months (median, 78 months). Side effects included transient bone marrow suppression, nausea, and fatigue. Secondary malignancy was encountered in one patient, whose carcinoma of the urinary bladder was treated successfully. Conclusions: Adequate immunosuppression with alkylating agents may favorably alter the long-term prognosis of patients with serpiginous choroiditis.