Scleritis is most common among women aged 30 to 50 years, and many have connective tissue diseases, such as rheumatoid arthritis, systemic lupus erythematosus, polyarteritis nodosa, granulomatosis with polyangiitis (formerly called Wegener granulomatosis), or relapsing polychondritis. A few cases are infectious in origin. About half of the cases of scleritis have no known cause. Scleritis most commonly involves the anterior segment and occurs in 3 types—diffuse, nodular, and necrotizing.
(See also Overview of Conjunctival and Scleral Disorders.)
Scleritis causes pain (often characterized as a deep, boring ache) severe enough to interfere with sleep and appetite. Photophobia and lacrimation may occur. Hyperemic patches develop deep beneath the bulbar conjunctiva and are more violaceous than those of episcleritis or conjunctivitis. The palpebral conjunctiva is normal. The involved area may be focal (usually one quadrant of the globe) or involve the entire globe and may contain a hyperemic, edematous, raised nodule (nodular scleritis) or an avascular area (necrotizing scleritis). Posterior scleritis is less common and is less likely to cause red eye but more likely to cause blurred or decreased vision.
In severe cases of necrotizing scleritis, perforation of the globe and loss of the eye may result. Connective tissue disease occurs in 20% of patients with diffuse or nodular scleritis and in 50% of patients with necrotizing scleritis. Necrotizing scleritis in patients with connective tissue disease signals underlying systemic vasculitis.
Rarely, nonsteroidal anti-inflammatory drugs are sufficient for mild cases of scleritis. However, usually a systemic corticosteroid (eg, prednisone 1 to 2 mg/kg orally once a day for 7 days, then tapered off by day 10) is the initial therapy. If inflammation returns, a longer dose of oral corticosteroid, also starting at prednisone 1 to 2 mg/kg orally once a day or pulsed intravenous corticosteroid, such as methylprednisolone 1000 mg IV daily for 3 days, can be considered. If patients are unresponsive to or intolerant of systemic corticosteroids or have necrotizing scleritis and connective tissue disease, systemic immunosuppression with cyclophosphamide, methotrexate, mycophenolate mofetil, or biologic agents (eg, rituximab, adalimumab) is indicated but only in consultation with a rheumatologist. Scleral grafts may be indicated for threatened perforation.
Scleritis is severe, destructive, vision-threatening inflammation.
Symptoms include deep, boring ache; photophobia and tearing; and focal or diffuse eye redness.
Diagnosis is made clinically and by slit-lamp examination.
Most patients require systemic corticosteroids and/or systemic immunosuppressive therapy, prescribed in consultation with a rheumatologist.
Scleral grafts may be indicated for threatened perforation.