Scleritis is most common among women aged 30 to 50 years, and many have systemic rheumatic diseases, such as rheumatoid arthritis Rheumatoid Arthritis (RA) Rheumatoid arthritis is a chronic systemic autoimmune disease that primarily involves the joints. Rheumatoid arthritis causes damage mediated by cytokines, chemokines, and metalloproteases.... read more , 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 , 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 (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 . 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 Overview of Conjunctival and Scleral Disorders The conjunctiva lines the back of the eyelids (palpebral or tarsal conjunctiva), crosses the space between the lid and the globe (forniceal conjunctiva), then folds back on itself as it spreads... read more .)
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 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
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.
Treatment of 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.
Pearls & Pitfalls
Prognosis for Scleritis
Of patients with scleritis, 14% lose significant visual acuity within 1 year, and 30% lose significant visual acuity within 3 years.
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.
Drugs Mentioned In This Article
|Drug Name||Select Trade|
|Deltasone, Predone, RAYOS, Sterapred, Sterapred DS|
|A-Methapred, Depmedalone-40, Depmedalone-80 , Depo-Medrol, Medrol, Medrol Dosepak, Solu-Medrol|
|Cyclophosphamide, Cytoxan, Neosar|
|RIABNI, Rituxan, RUXIENCE, truxima|
|AMJEVITA , Humira|