Scleritis is a severe, destructive, vision-threatening inflammation involving the deep episclera and sclera. Symptoms are moderate to marked pain, hyperemia of the globe, lacrimation, and photophobia. Diagnosis is clinical. Treatment is with systemic corticosteroids and possibly other immunosuppressants.
Scleritis is most common among women aged 30 to 50 years, and many have systemic rheumatic 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.)
Symptoms and Signs of Scleritis
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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 (diffuse scleritis) and may contain a hyperemic, edematous, raised nodule (nodular scleritis) or an avascular area (necrotizing scleritis). Intermediate scleritis and posterior scleritis are less common and are less likely to cause red eye but more likely to cause floaters, blurring, or decreased vision.
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In severe cases of necrotizing scleritis, perforation of the globe and loss of the eye may result. Systemic rheumatic disease occurs in 20% of patients with diffuse or nodular scleritis and in 50% of patients with necrotizing scleritis. Necrotizing scleritis in patients with systemic rheumatic disease signals underlying systemic vasculitis. Clinicians should carefully consider infection in patients with necrotizing scleritis, because infectious scleritis may manifest similarly and because infection may complicate necrotizing scleritis.
Diagnosis of Scleritis
Clinical evaluation
Diagnosis of scleritis is made clinically and by slit-lamp examination, which typically reveals a scleral violaceous hue that originates deep to the conjunctiva. Smears or rarely biopsies are necessary to confirm infectious scleritis. CT or ultrasonography may be needed for posterior scleritis.
Treatment of Scleritis
Systemic corticosteroids
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 10 mg each week as tolerated or as indicated based on the clinical picture) 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 systemic rheumatic 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.
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Prognosis for Scleritis
Of patients with scleritis, 14% lose significant visual acuity within 1 year, and 30% lose significant visual acuity within 3 years.
Key Points
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.
