Scleritis

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 Episcleritis Episcleritis is self-limiting, recurring, usually idiopathic inflammation of the episcleral tissue that does not threaten vision. Symptoms are a localized area of hyperemia of the globe, irritation... read more or conjunctivitis Overview of Conjunctivitis Conjunctival inflammation typically results from infection, allergy, or irritation. Symptoms are conjunctival hyperemia and ocular discharge and, depending on the etiology, discomfort and itching... read more . 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.

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 Overview of Vasculitis Vasculitis is inflammation of blood vessels, often with ischemia, necrosis, and organ inflammation. Vasculitis can affect any blood vessel—arteries, arterioles, veins, venules, or capillaries... read more . 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 is made clinically and by slit-lamp examination Slit-lamp examination The eye can be examined with routine equipment, including a standard ophthalmoscope; thorough examination requires special equipment and evaluation by an ophthalmologist. History includes location... read more , 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.

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.

Of patients with scleritis, 14% lose significant visual acuity within 1 year, and 30% lose significant visual acuity within 3 years.