Fulminant proliferative vitreoretinopathy in syphilitic uveitis

BACKGROUND: Syphilis is a reemerging sexually transmitted disease that can lead to any type of intraocular inflammation. Prognosis of syphilitic uveitis after appropriate therapy is classically regarded as favorable. However, visual threatening complications may develop, rarely including rhegmatogenous/tractional retinal detachment (R/T RD) and proliferative vitreoretinopathy. FINDINGS: We report 4 patients presenting with complex R/T RD and fulminant proliferative vitreoretinopathy despite treatment among 19 patients with syphilitic posterior uveitis consecutively seen at our uveitis service. Most of these complications occurred during or shortly after antibiotic therapy. All patients presented with significant intraocular inflammation, including vitritis, occlusive retinal vasculitis, and retinal infiltrates (necrotizing retinochoroiditis in six eyes of four patients). Two patients (50 %) tested HIV positive, and the same proportion had inadvertently received high dose oral ± intravenous corticosteroids prior to diagnosis of syphilis. Two patients (three eyes) underwent RD surgical repair. Histopathology of an excised epiretinal membrane disclosed fibroglial tissue, with immature glial cells and metaplastic retinal pigment epithelium, admixed with lymphoplasmacytic infiltrate. CONCLUSIONS: Syphilitic uveitis may be complicated by complex RD/fulminant fibroglial proliferation, occurring during/after treatment. Predisposing factors are currently unknown but may include prior use of corticosteroid, necrotizing retinitis and/or high spirochaetal load. A significant inflammatory component may underlie this fulminant fibroglial proliferation, being possibly amenable to modulation by aggressive anti-inflammatory therapy delivered concurrently with parenteral antibiotics.