Isolated ANCA-associated scleritis successfully treated with systemic rituximab; a case report and review of literature.

Abstract

Background: Necrotizing scleritis is one of the most destructive ocular manifestations of underlying systemic diseases that can lead to a variety of severe complications, including globe perforation or vision loss. Necrotizing scleritis can occur in various conditions, such as systemic vasculitis like Anti-Neutrophil Cytoplasmic Antibody (ANCA) and ANCA-Associated vasculitis (AAV), systemic autoimmune disorders, infections, or as a result of surgical procedures.

Case presentation: In this case report, we present a patient with acute bilateral isolated necrotizing sclerokeratitis associated with positive c-ANCA without any manifestation of other organ involvement. A 52-year-old man with acute anterior bilateral necrotizing scleritis with diffuse areas of necrosis, thinning of the sclera, and the choroidal show was observed which was impending perforation on both sides. Systemic medications, including intravenous methylprednisolone and cyclophosphamide, and topical medications were administered. However, due to an incomplete response to these drugs, intravenous rituximab was initiated. Significant improvements in clinical manifestations were initiated after treatment with Rituximab.

Conclusion: Our case highlights the importance of systemic rituximab therapy in treating isolated ANCA-associated necrotizing scleritis when initial immunosuppressive treatments are not fully effective.