Case report peripheral ulcerative keratitis with nodular scleritis, presents as the primary manifestation of granulomatosis with polyangiitis.

Granulomatosis with polyangiitis (GPA) is an ANCA-associated autoimmune disorder that typically affects small and/or medium-sized blood vessels. Being a life-threatening disease, with timely suspicion, targeted laboratory investigations, and collaborative management from the ophthalmologist and rheumatologist led to long-term remission of the disease. Case: A 38-year-old female complaining of recurrent deep boring pain with redness in her left eye for many years, which was diagnosed as nodular scleritis with peripheral ulcerative keratitis. The patient also has recurrent bouts of epistaxis and in suspicion of GPA laboratory investigations were carried out and later diagnosed. She was started on cyclophosphamide and is currently under maintenance with rituximab. Case discussion: Ocular involvement has been shown to occur in 20-50% of the population in several studies. It causes conjunctivitis, episcleritis, scleritis, necrotizing keratitis, corneoscleral perforation, posterior uveitis, and optic neuritis. The positive C-ANCA and high PR3 autoantibody have high sensitivity and association with GPA. Cyclophosphamide has been shown to be an effective treatment in multiple studies whereas rituximab has been emerging as a new treatment modality for maintenance, which in turn helps in the remission and relapse of GPA cases. Conclusion: Scleritis and peripheral ulcerative keratitis can be the manifestation of a GPA. Careful evaluation, diagnosis, and management with a multidisciplinary medical team, early start of cyclophosphamide, and rituximab have a huge role in decreasing the disease activity and is life-saving.