Presumed granulomatosis with polyangiitis presenting with anterior scleritis and inflammatory ciliary body granuloma.

Purpose: To present a case of presumed limited granulomatosis with polyangiitis (GPA) associated with anterior scleritis and ciliary body inflammatory granuloma which was treated with systemic rituximab (RTX), oral mycophenolate mofetil, and intravitreal (IVT) dexamethasone implant.

Observations: We report a patient presenting with sectoral scleritis and ciliary body granuloma in the left eye. The patient also had a nasal sinus granuloma which was biopsied three times with negative results for malignancy and fungal infections. The patient underwent a diagnostic vitrectomy, which was also negative for lymphoma, bacterial and fungal infections. Subsequently, intravenous methylprednisolone and oral methotrexate were started, but significant improvement was achieved only following initiation of intravenous RTX, oral mycophenolate mofetil, and IVT dexamethasone implant.

Conclusion: Therapeutic management of scleritis associated with limited GPA can be very challenging; early diagnosis can help to eliminate potential complications. Our result showed that RTX, mycophenolate mofetil, and IVT dexamethasone implant can be beneficial in treatment-resistant cases.