Abstract

Scleritis is a rare, vision-threatening inflammation of the sclera that is often associated with life-threatening systemic illnesses. Rheumatoid arthritis remains the most common associated systemic rheumatic disease and the commonest systemic association of scleritis. Granulomatosis with polyangiitis is the most common cause of vasculitis-associated scleritis. The etiopathogenesis of scleritis remains unclear, but can be immune complex-mediated or due to a local delayed hypersensitivity reaction. Scleritis can involve either the anterior or posterior sclera, and has a wide spectrum of clinical presentations. Among the subtypes of scleritis, necrotizing scleritis has an increased risk of complications and is more commonly associated with anterior uveitis and peripheral ulcerative keratitis. Posterior scleritis is often not diagnosed or missed due to its subtle clinical signs and protean manifestations. Meticulous history taking, detailed ocular examination, and a targeted array of investigations with a multi-disciplinary approach to find any underlying systemic disease are crucial for the management of a case of scleritis. Corticosteroids remain the mainstay of short-term treatment of scleritis; mild to moderate scleral inflammation may respond well to treatment with nonsteroidal antiinflammatory drug or topical corticosteroid. Corticosteroid-sparing immunosuppressive therapies are useful in cases with an inadequate response or failure to provide long-term control of inflammation, and to prevent recurrence of scleritis. Biologic agents are increasingly used in the management of scleritis, not responding to the conventional therapies. This review provides an overview of the various subtypes of scleritis and its systemic associations and evaluates current trends in the diagnosis and management of noninfective scleritis.

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