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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
immunosuppressants.
Scleritis is most common among women aged 30 to 50 yr, and many have connective tissue diseases, such as RA, SLE, polyarteritis nodosa, Wegener's 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 (scleromalacia perforans).
Symptoms and Signs
Pain (often characterized as a deep, boring ache) is 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 an underlying systemic vasculitis.
Diagnosis
Diagnosis is made clinically and by slit-lamp examination. Smears or rarely biopsies are necessary to confirm infectious scleritis. CT or ultrasound may be needed for posterior scleritis.
Prognosis
Of patients with scleritis, 14% lose significant visual acuity within 1 yr and 30% lose significant visual acuity within 3 yr. Patients with necrotizing scleritis and underlying systemic vasculitis have a mortality rate of up to 50% in 10 yr (mostly from MI).
Treatment
Occasionally, NSAIDs are sufficient for mild cases. However, usually a systemic corticosteroid (eg, prednisone 1 mg/kg po once/day) is the initial therapy. If scleritis is unresponsive to or intolerant of systemic corticosteroids or the patient has necrotizing scleritis and connective tissue disease, systemic immunosuppression with cyclophosphamide or azathioprine is indicated, but only in consultation with a rheumatologist. Scleral grafts may be indicated for threatened perforation.
Last full review/revision June 2008 by Mitchell H. Friedlaender, MD
Content last modified June 2008
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