Conditions

What is scleritis?

Scleritis is an uncommon inflammation of the sclera, the tough, white, structural wall of the eyeball.

Swelling and inflammation of sclera can occur in one or both eyes, can affect surrounding tissues, and be quite dramatic and dangerous to vision.

Scleritis is common in patients that have an underlying autoimmune disease (e.g. rheumatoid arthritis) or other disease process.

Scleritis typically occurs in patients 30-60 years old and is rare in children.

Scleritis must be differentiated from episcleritis, a much less severe inflammation that affects the episclera, the thin outside layer of the sclera.

Episcleritis is normally neither as severe nor characteristic as scleritis.

What are the signs and symptoms?

Scleritis typically manifests with red and painful eye. Redness may be restricted to a specific area of the eye, or widespread.

Pain can be severe, mostly felt as a deep pain around the eye, which can radiate to the ears and jaw, tends to get worse drying the night, many times waking the patient up and can be made worse when moving the eye.

Vision can be affected if swelling from inflammation affects surrounding tissues such as lens, cornea, choroid, retina, or optic nerve.

Common symptoms of scleritis include:

Gradual onset of pain leading to fairly severe eye pain that may spread to the surrounding head
Redness on the white part of the eye - can involve a small sector or the entire white of the eye
Decrease in vision
If the posterior part of the eye is involved, it can be associated with inflammation of the socket and produce double vision and/or pain upon eye movement
Recurrent/chronic symptoms in patients with underlying systemic disease

Common signs of scleritis include:

Inflammation (swelling) of the sclera, episclera, and overlying conjunctival blood vessels
Red patches do not blanch (whiten) with topical phenylephrine (decongestant eye drop)
Thinning of the sclera can be seen after severe disease and leaves a darker area where the inflammation occurred (white layer appears blue because underlying tissue, the choroid, becomes visible)

Causes of scleritis

In 50% of patients, scleritis is associated with an underlying systemic disease process.

Common associated systemic diseases include autoimmune diseases (rheumatoid arthritis, systemic lupus erythematosus, inflammatory bowel syndrome, etc), herpes zoster (shingles), syphilis and gout.
Scleritis is very frequently idiopathic, meaning that no cause is found.

Diagnosis

If these symptoms are experienced, the patient should have a complete eye exam including dilation to rule out other disorders. Diagnosis is based on the presence of characteristic clinical features and symptoms.

Underlying systemic disease is the cause in about 50% of cases so a physical exam with their primary care physician including a blood work-up for auto-immune disease

Treatment

Treatment should be concentrated at reducing inflammation quickly.

Antibiotic therapy can be used when an infectious cause is known or even suspected. Infections of the sclera are uncommon and are usually related to previous surgery or trauma.

For non-infectious causes of scleritis, which are by far the most common, treatment may include oral non-steroidal anti-inflammatory (NSAIDs), oral corticosteroids, and/or oral immunosuppressive therapy.

Oral non-steroidal anti-inflammatory drugs (NSAIDs) are mostly used as the first line of therapy in milder cases. Intravenous steroids can be used in very acute cases.

Of course, dependence on steroid therapy should be avoided due to complications of long-term steroid use.

Immunomodulatory therapy can be effectively added for non-infectious scleritis to allow long term control of inflammation and minimize the side effects of the steroids, with regular monitoring for blood work and side effects.

Length of treatment and follow up will depend on the severity of the signs and symptoms.

Surgery to repair perforated sclera, or strengthen dangerously thinned sclera, can be done with prepared scleral grafts, or other similar available sterile tissue.

Complications and prognosis

If the scleritis and underlying disease are not treated promptly, the inflammation can spread to other parts of the eye.

Also, symptoms can recur, and more serious complications can develop.

Scleritis can worsen and progress to ischemia and necrosis, which may lead to scleral thinning and perforation of the globe and possibly lead to blindness.

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