Ocular clues to a systemic disease: A case report of granulomatosis with polyangiitis, presenting with scleral thinning and peripheral ulcerative keratitis

Granulomatosis with polyangiitis (GPA) (formerly Wegener’s granulomatosis) is a systemic necrotising, granulomatous vasculitis affecting small- to medium-sized blood vessels. Although GPA most commonly involves the respiratory tract and kidneys, ocular manifestations are reported in up to 50% of cases, with scleritis and peripheral ulcerative keratitis (PUK) being notable presentations. Previously a uniformly fatal disease, treatment with cytotoxic and immunosuppressive agents has greatly improved the outcome and survival. We report the case of a 47 year old female diagnosed with systemic GPA with scleral thinning and uveal show, who later developed PUK. This case underscores the importance of recognising ocular signs, such as scleritis and PUK, as potential initial manifestations of systemic GPA. Early diagnosis and a multidisciplinary approach involving medical and surgical interventions are crucial for preserving vision and preventing disease progression.