Definition The sclera is the white, outer coat of the eye. When this area becomes inflamed, the condition is called scleritis.
What is going on in the body? The sclera, which is made up of protein and connective tissue, becomes inflamed and irritated. If untreated, the disease may last a long time. Even with proper treatment, it is likely to recur. Complications from the disease can cause serious eye damage or loss of the eye.
What are the signs and symptoms of the disease? Symptoms include:
redness and puffiness of the eye
engorgement of blood vessels
tearing
eye pain and tenderness
sensitivity to light
blurred vision
What are the causes and risks of the disease? Once in a while the sclera may become infected with bacteria or other organisms, such as syphilis. However, most cases are linked to some form of collagen disease. Collagen is a type of protein found in many areas of the body, including the sclera, skin, joints, and blood vessels. In collagen diseases, the body's immune system attacks the person's own body, primarily affecting the areas containing collagen. People with scleritis often have inflammation in the skin, joints, or blood vessel walls. Examples of common collagen diseases that may cause scleritis are rheumatoid arthritis and lupus.
The severity of the disease varies. It can appear as a mild inflammation. A more serious form of the condition is known as nodular scleritis, which involves clusters of cells. The most severe type is necrotising scleritis, which causes tissue death. There is a poor chance of recovery from this condition both for the affected eye and for the individual's overall health. People with necrotising scleritis often do not live longer than 5 years. Scleritis most often occurs between the ages of 30 and 50. Women are more prone to develop the condition. About 50% of the time, it affects both eyes.
In half of the people with scleritis, the condition is associated with some form of systemic disease, which is an illness involving many body systems. For this reason, a thorough physical examination is important to diagnosis. Often the eye specialist works together with a rheumatologist in managing this condition.
What can be done to prevent the disease? There are no known effective means to prevent scleritis.
How is the disease diagnosed? The history often helps suggest the diagnosis and the most notable feature of scleritis is pain in the eye. This is what usually prompts a person to seek medical care. The pain may be felt in the temple, jaw, or sinuses. It is usually severe enough to prevent a person from sleeping. Analgesia is usually only partially effective. The individual may also report tearing and sensitivity to light. The opthalmologist (eye doctor) will observe the eye under a special microscope. The inflammation of the eye will be easily seen and the diagnosis can be made. Sometimes, the eye also has a bluish-red hue.
What are the treatments for the disease? The primary goal of treatment is to prevent the loss of vision. Local corticosteroid therapy can be used to ease the person's discomfort. However, this is usually not enough to eliminate the condition. Most cases require non-steroidal anti-inflammatory drugs. Oral corticosteroid may also be used. Once the condition is under control, the dose of corticosteroids may be reduced. It may be necessary for the individual to continue on a low level of the medication until the condition resolves naturally. Rarely, intravenous (IV) hydrocortisone may be required.
What are the side effects of the treatments? High doses of corticosteroids may cause a cataract, which is a clouding of the lens of the eye that causes vision problems, and inflammation of the cornea, the thin membrane that covers the central, coloured part of the eye.
What happens after treatment for the disease? Complications may occur as the disease progresses. These vary with the amount of inflammation. Over time, the disease alters the sclera. It becomes more transparent, letting the underlying pigment show through. This gives the eye a darker appearance. Other complications include:
glaucoma, or increased pressure within the eyeball that may lead to vision loss
retinal detachment, or separation of the retina from the back of the eyeball
swelling of the optic nerve, which carries messages from the eye to the brain
uveitis, or inflammation of the coloured structures in the eye
Unfortunately, as many as 25% of individuals with this condition lose a significant portion of their vision within a year of getting the condition. This occurs despite active therapy.
Author: William Stevens, MD Reviewer: HealthAnswers Australia Medical Review Panel Editor: Dr David Taylor, Chief Medical Officer HealthAnswers Australia Last Updated: 1/10/2001 Contributors Potential conflict of interest information for reviewers available on request
