Serpiginous choroiditis (SC) is a rare, chronic, asymmetrically bilateral posterior uveitis with a recurrent course. The exact prevalence of SC is unknown; however, it is estimated between 1.6% and 5.3% of posterior uveitis cases in non-endemic areas for tuberculosis. Major features of SC include choriocapillaris occlusive vasculitis, secondary endothelial cell injury, and subsequent atrophy of the retinal pigment epithelium, outer retina, and choroid. The prognosis of vision is poor when it involves the macula, especially in the macular variant of SC. Moreover, the macular variant has a higher risk of developing choroidal neovascularization in nearly half of the affected patients.
There are no controlled trials for the treatment of SC due to the rarity of the disease. Cytosine arabinoside, azathioprine, and oral prednisone were used with reported improved visual acuity in one month. Employment of a combination of cyclosporine and oral prednisone as a treatment of active SC had conflicting results. Hopper and Kaplan reported a triple-agent regimen of azathioprine, cyclosporine, and oral prednisone that resulted in rapid control of the active SC and vision recovery; however, disease recurrence was the study’s main problem.
We studied the efficacy of immunosuppressive treatment in patients with serpiginous choroiditis by analyzing the clinical course of 12 eyes from 6 patient six patients with vision threatening, steroid dependent or steroid resistant serpiginous choroiditis treated with a combination of immunosuppressive agents, including azathioprine (AZA), cyclosporin A CsA), or cyclophosphamide. All patients underwent treatment for at least 12 months.
The average follow-up period was 57 months (range: 17-105 months). All patients were able to taper oral steroids. All six patients have discontinued all immunosuppressive medication after a treatment period of 12 to 19 months. Ten eyes showed improved visual acuity, while vision remained stable in two eyes due to macular scarring. Recurrence occurred in two patients when attempts were made to reduce the dose of immunosuppressive medications before the one-year target. Two patients experienced side effects that resolved after reducing the medication dose.
Since recent literature shows that alkylating agents are still the most potent and effective treatment in patients with serpiginous choroiditis. We studied and demonstrated the efficacy of chlorambucil in achieving long-term remission and possible cure in patients with serpiginous choroiditis.
In conclusion, long-term immunosuppressive treatment appears to prolong remission and preserve vision in patients with serpiginous choroiditis.
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