Necrotizing Scleritis and Peripheral Ulcerative Keratitis Associated with Wegener’s Granulomatosis

INTRODUCTION: To evaluate the complications, efficacy of medical and surgical treatment, and outcome in patients with necrotizing scleritis and peripheral ulcerative keratitis associated with Wegener’s granulomatosis. METHODS: The authors reviewed a series of seven patients with Wegener’s granulomatosis treated in the Corneal Department of Zhongshan Ophthalmic Center and the Department of Ophthalmology of Kashgar First People’s Hospital. A detailed chart review was performed to determine demographic characteristics, ocular presentation, biopsy and laboratory testing results, treatment, and final outcome. RESULTS: Wegener’s granulomatosis was indicated by ocular and/or systemic findings; biopsy and immunohistochemistry results supported the diagnosis. Patients with necrotizing scleritis and/or peripheral ulcerative keratitis received cytotoxic immunosuppressive therapy; this, in conjunction with surgical treatment, halted the relentlessly progressive inflammation and preserved the integrity of the globe in 78% of eyes. Best-Corrected Visual Acuity remained stable in four of nine eyes, was improved in two of nine eyes, and decreased in three of nine eyes (secondary to cataract and/or stromal scarring). Although one patient died, treatment with corticosteroids and cytotoxic agents dramatically improved outcomes in these patients. CONCLUSION: Necrotizing scleritis and peripheral ulcerative keratitis often have a poor visual outcome, and may herald an underlying systemic vasculitis. Wegener’s granulomatosis, with the associated necrotizing scleritis and peripheral ulcerative keratitis, should be managed with aggressive immunosuppression to avoid the associated morbidity and mortality. Thus, the ophthalmologist may play a significant role in its early diagnosis and treatment.