MSD Manual

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Melvin I. Roat

, MD, FACS, Sidney Kimmel Medical College at Thomas Jefferson University

Last full review/revision Apr 2021
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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 years, and many have connective tissue diseases, such as rheumatoid arthritis Rheumatoid Arthritis (RA) Rheumatoid arthritis (RA) is a chronic systemic autoimmune disease that primarily involves the joints. RA causes damage mediated by cytokines, chemokines, and metalloproteases. Characteristically... read more Rheumatoid Arthritis (RA) , systemic lupus erythematosus Systemic Lupus Erythematosus (SLE) Systemic lupus erythematosus is a chronic, multisystem, inflammatory disorder of autoimmune etiology, occurring predominantly in young women. Common manifestations may include arthralgias and... read more Systemic Lupus Erythematosus (SLE) , polyarteritis nodosa Polyarteritis Nodosa (PAN) Polyarteritis nodosa is a systemic necrotizing vasculitis that typically affects medium-sized muscular arteries and occasionally affects small muscular arteries, resulting in secondary tissue... read more , granulomatosis with polyangiitis Granulomatosis with Polyangiitis (GPA) Granulomatosis with polyangiitis is characterized by necrotizing granulomatous inflammation, small- and medium-sized vessel vasculitis, and focal necrotizing glomerulonephritis, often with crescent... read more Granulomatosis with Polyangiitis (GPA) (formerly called Wegener granulomatosis), or relapsing polychondritis Relapsing Polychondritis Relapsing polychondritis is a rare, episodic, inflammatory, and destructive disorder involving primarily cartilage of the ear and nose but also potentially affecting the eyes, tracheobronchial... read more 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.

Symptoms and Signs of 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 Episcleritis 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 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.

Diagnosis of Scleritis

  • Clinical evaluation

Diagnosis of scleritis is made clinically and by slit-lamp examination. Smears or rarely biopsies are necessary to confirm infectious scleritis. CT or ultrasonography may be needed for posterior scleritis.

Prognosis for Scleritis

Of patients with scleritis, 14% lose significant visual acuity within 1 year, and 30% lose significant visual acuity within 3 years. Patients with necrotizing scleritis and underlying systemic vasculitis have a mortality rate of up to 50% in 10 years (mostly due to myocardial infarction).

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 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.

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.

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