Reiter's Syndrome

Definition

Uveitis is an inflammation of the uveal tract, which lines the inside of the eye behind the cornea. Much of the uvea lies between the retina and tough, outer sclera. The uveal tract has three parts: the iris, the ciliary body, and the choroid. Uveitis is categorized according to the part of the uveal tract that is affected. Anterior uveitis is an inflammation of the front part of the uveal tract; it includes inflammation of the iris (iritis) and inflammation of the iris and the ciliary body (iridocyclitis). Posterior uveitis is an inflammation of the part of the uveal tract behind the lens of the eye. It includes inflammation of the choroid (choroiditis) and inflammation of the choroid and retina (chorioretinitis). Uveitis that affects the entire uveal tract is called panuveitis or diffuse uveitis.

Description

The uveal tract is made up of the iris, ciliary body, and choroid. The iris is the colored part of the eye. The ciliary body in inside the eye and produces a fluid called aqueous humor. Ciliary muscles aid in accommodation, the process of changing the shape of the lens in the eye to see things at various distances. The choroid lines the back of the eye and has many blood vessels. It helps nourish part of the retina. The choroid lies between the retina and outermost sclera.

Uveitis may either persist for a long time (chronic) or have a short-term duration (acute). Anterior uveitis is classified as either granulomatous or nongranulomatous. The distinction is based on the disease agents that were considered responsible for the condition. At one time, it was thought that granulomatous uveitis was caused by tuberculosis bacilli whereas nongranulomatous uveitis was thought to be caused by streptococci. The distinction is still used even though the causes of uveitis are now understood differently.

In most cases, uveitis affects only one eye, although posterior uveitis sometimes involves both eyes. About 60% of cases develop within the eye itself, but 40% are associated with systemic diseases or disorders ranging from toxoplasmosis to syphilis. Many of these are diseases of childhood and adolescence. Uveitis does not appear to run in families or to be associated with lifestyle choices, occupational history, geographical location, or environmental factors.

Uveitis is a serious condition that may develop rapidly and cause lasting damage to the eye. Patients who think they may have chronic uveitis should seek evaluation and treatment by an ophthalmologist (a physician who specializes in diseases of the eye) as soon as possible. If the patient has a sudden loss of vision and the eye looks inflamed, the patient should go immediately to the doctor for emergency treatment.

Causes & symptoms

The causes of uveitis are not fully understood, but they can be a result of trauma, allergy, or a response to a systemic or ocular disease. Uveitis may be a type of immune-response mechanism. In people with impaired immune systems, uveitis may be due to an infection.

Chronic uveitis is often associated with systemic disorders (e.g., Lyme disease, sarcoidosis, or juvenile rheumatoid arthritis).

Anterior uveitis

The so-called classic symptoms of anterior uveitis--severe pain; redness, particularly around the edge of the iris; and extreme sensitivity to light (photophobia)--occur mostly in acute uveitis. In anterior uveitis, the doctor will see a so-called "flare and cell" pattern when looking into the watery fluid (aqueous humor) between the cornea and the lens of the patient's eye. The iris may adhere to the lens, thus increasing the intraocular pressure. There may be nodules on the iris. There may be tearing and the pupil may be constricted and nonreactive. In severe cases of anterior uveitis, there may be hypopyon (a small amount of pus or collection of white cells) visible when the doctor examines the eye.

In granulomatous uveitis, there will be large yellowish-white cells visible on the back of the cornea, and possibly some small nodules on the iris. Granulomatous uveitis is usually less acute than the nongranulomatous form; the eye is only mildly inflamed and the patient's vision is somewhat blurred.

Granulomatous uveitis can be produced by syphilis, toxoplasmosis, cytomegalovirus, sarcoidosis, tuberculosis, or Vogt-Koyonagi-Harada syndrome (VKH). VKH is marked by severe uveitis associated with hair loss, hearing loss, loss of pigment in the eyelashes and brows, and headaches. It occurs most commonly in Asians.

In nongranulomatous uveitis, the cells visible on the cornea are smaller, and there are no masses on the iris. This type of anterior uveitis is, however, more painful. The eye is red and the patient experiences both photophobia and loss of vision.

Systemic diseases that can cause nongranulomatous uveitis include ankylosing spondylitis, Reiter's syndrome, psoriasis, ulcerative colitis, Behcet's syndrome, Lyme disease, and Crohn's disease. Children--especially girls--with anterior uveitis should be screened for juvenile rheumatoid arthritis (JRA).

Posterior uveitis

The symptoms of posterior uveitis are sometimes subtle. The patient may notice blurred or hazy vision, or floating black spots before the eyes. There may be pain and photophobia. The iris may attach to the lens in the eye thus increasing intraocular pressure.

Posterior uveitis may be acute or chronic. It is more likely to involve both eyes. When the doctor examines the eye, cells may be seen in the vitreous humor, which is the normally transparent gel that fills the eyeball behind the lens. There will be yellowish or dark areas of inflammation on the choroid and the retina. The blood vessels in the retina develop a sheath or covering of inflammatory tissue. In severe cases, the vitreous humor is so cloudy that the doctor cannot see the retina at the back of the eye.

Pars planitis is an inflammation of the pars plana, which is a part of the ciliary body. Pars planitis usually occurs in older children or young adults, and can develop into posterior uveitis.

The diseases that cause granulomatous uveitis may also cause posterior uveitis.

Diagnosis

The eye doctor will examine the patient's eyes with a slit lamp in order to rule out conjunctivitis and certain types of glaucoma. The slit lamp is an instrument that combines a binocular microscope with a special light. The slit lamp can shine a narrow beam of very bright light into the eye and allow the doctor to examine the front part of the eye in detail. The slit-lamp exam is not painful, however if the patient is sensitive to light there will be discomfort.

The absence of a discharge from the eye and the absence of infectious organisms in a laboratory smear usually rule out conjunctivitis. In addition, the size of the pupil is often small in uveitis whereas it is normal in conjunctivitis. In acute glaucoma, the patient has severe pain, the cornea of the eye is cloudy, and the pressure level of the fluid inside the eye is abnormally high; whereas in uveitis the pain is moderate, the cornea is clear, and the fluid pressure is normal or possibly lower or slightly above normal. The doctor may also use the slit lamp and another lens to examine the back of the eye to get a good look at the retina and choroid. Other instruments, such as a hand-held ophthalmoscope or a binocular indirect ophthalmoscope, can be used to examine the back of the eye. There should be no discomfort with these tests except if the patient is sensitive to the bright light.

Laboratory testing

Laboratory testing is used to rule out conjunctivitis in some patients. The doctor wipes the inside of the patient's eyelid with a swab in order to obtain a sample for testing. Although blood tests are not necessary to diagnose uveitis by itself, they are used to diagnose the cause if the doctor suspects that toxoplasmosis or another systemic disease is responsible for the uveitis.

Treatment

Uveitis is generally treated by an ophthalmologist because therapy requires topical and oral medications, however, some optometrists (O.D.) are state licensed to use therapeutic medications. Other doctors may be involved in treating the underlying disease, if the patient has one, and in monitoring the patient's responses to medications.

Anterior uveitis is treated with corticosteroid drops; in severe cases, the patient may be given steroid injections in the area of the eye or oral steroids. Atropine sulfate drops may be given to dilate the patient's pupil. Posterior uveitis is treated with systemic corticosteroids. It is usually not necessary to dilate the pupil.

Prolonged steroid use may increase intraocular pressure, thereby increasing the risk of glaucoma. Steroid use has also been connected to cataract formation. Patients should be monitored closely and frequently.

Prognosis

The prognosis depends upon the location of the uveitis, on whether it is chronic or acute, and on the promptness of treatment. The prognosis for untreated uveitis is poor. Untreated anterior uveitis usually progresses to posterior uveitis, resulting in cataracts, scar tissue, and eventual glaucoma. If treated promptly, anterior uveitis usually clears up in several days or weeks, but is likely to recur. Posterior uveitis usually results in some permanent loss or blurring of vision.

Prevention

Patients with anterior uveitis should be warned about the possibility of recurrence and instructed about its symptoms, especially inflammation of the iris. They should be advised to seek treatment at once at the first signs of recurrence.

Key Terms

Choroid

The part of the uveal tract behind the ciliary body. The choroid underlies and nourishes the retina and absorbs scattered light.

Ciliary body

The part of the uveal tract between the iris and the choroid.

Cornea

The transparent front part of the eye that covers the iris and pupil.

Flare and cell

A pattern revealed by slit-lamp examination that indicates uveitis. Flare and cell resembles light filtered through smoke.

Hypopyon

A small amount of pus or collection of white cells that is visible in the front of the eye in severe cases of anterior uveitis.

Iris

The circular membrane that forms the colored portion of the eye and expands or contracts around the pupil.

Photophobia

Extreme sensitivity to light. Photophobia is a major symptom of acute uveitis.

Pupil

The opening in the center of the iris that allows light to pass through to the retina.

Retina

The innermost membrane at the back of the eyeball on which images are projected by the lens.

Slit lamp

An instrument that combines a binocular microscope with special lights. It allows an eye doctor to examine the front portion of the eye.

Uveal tract

The pigmented membrane that lines the back of the retina of the eye and extends forward to include the iris. The uveal tract is sometimes called the uvea and has three parts: the iris, the choroid, and the ciliary body.

Vitreous humor

The clear gel-like substance that fills the eyeball behind the lens.

Further Reading

For Your Information

Books

• Eisenbaum, Allan M. "Eye." In Current Pediatric Diagnosis & Treatment, edited by William W. Hay Jr., et al. Stamford, CT: Appleton & Lange, 1997.
• "Ophthalmologic Disorders: Uveal Tract; Uveitis." In The Merck Manual of Diagnosis and Therapy, edited by Robert Berkow, et al. Rahway, NJ: Merck Research Laboratories, 1992.
• Riordan-Eva, Paul, et al. "Eye." In Current Medical Diagnosis & Treatment 1998, edited by Lawrence M. Tierney Jr., et al. Stamford, CT: Appleton & Lange, 1998.
• Springhouse Corporation. Illustrated Guide to Diagnostic Tests. Springhouse, PA: Springhouse Corporation, 1994.
• "Uveitis." In Professional Guide to Diseases, edited by Stanley Loeb, et al. Springhouse, PA: Springhouse Corporation, 1991.

  Organizations

• American Academy of Ophthalmology. P.O. Box 7424, San Francisco CA. 94120-7424. (415) 561-8500. http://www.eyenet.org.
• American Optometric Association. 243 North Lindbergh Blvd., St. Louis, MO 63141. (314) 991-4100. http://www.aoanet.org.

Gale Encyclopedia of Medicine. Gale Research, 1999.