Information on the following diseases can be found in the Related Disorders section of this report:

• None

The initial symptoms of Reiter's Syndrome include painful urination due to inflammation of the tubes that carry urine from the kidneys to the bladder (urethritis). Other early symptoms may include abdominal pain, cramping, and diarrhea because of inflammation of the small intestine (enteritis). Painful and swollen joints (arthritis), as well as other symptoms, may appear 4 days to 4 weeks later. The initial symptoms of Reiter's Syndrome may go unreported and affected individuals may not seek out medical assistance until they experience painful arthritis.

The symptoms of urinary tract inflammation associated with Reiter's Syndrome may include pain while urinating, blood in the urine (hematuria), and/or pus in the urine. Pain in the joints, which occurs because of infection in other parts of the body (reactive arthritis), usually begins abruptly and effects more than one joint. Symptoms may include warm, red, swollen, and painful joints. The major joints of the legs and feet are most frequently affected. However, both sides of the body are usually not affected (asymmetric arthritis). The small joints of the hands are rarely involved. These arthritic symptoms usually last 2 to 4 months but may begin to ease after 2 to 6 weeks. Frequently, total remission during treatment occurs within the first year. However, some attacks may last for several years and the joints involved may become permanently damaged.

Other symptoms of Reiter's Syndrome that do not involve the joints (extraarticular) may include inflammation of tendons (tendinitis) which often affects the Achilles tendon at the back of the ankles, and foot pain associated with inflammation of the connective tissue in the feet (plantar fasciitis). Eventually the toes may have an appearance that resembles a sausage (dactylitis). Painful inflammation may also occur where muscles and tendons attach to bones (enthesitis). Approximately 10 to 20 percent of affected individuals also develop chronic pain and inflammation in the spine, shoulders, neck, and/or ribs (ankylosing spondylitis). (For more information on this disorder, choose "Ankylosing Spondylitis" as your search term in the Rare Disease Database.)

People with Reiter's Syndrome also experience redness and inflammation of the membranes that line the eyes (conjunctivitis). It is often mild, effects both eyes, and lasts for a few days. However, it often recurs. Occasionally, part of the eye (uvea) also becomes inflamed (uveitis or iritis), and symptoms may include increased sensitivity to light (photophobia). Approximately 5 percent of people with Reiter's Syndrome develop abnormally high fluid pressure within the eyes (glaucoma), a clouding of the lens of the eyes (cataracts), and diminished vision which can progress to blindness. (For more information on this disorder, choose "Cataracts" as your search term in the Rare Disease Database.)

People with Reiter's Syndrome may also develop lesions in the blood vessel rich layers of the skin or the mucous membranes (mucocutaneous vesicular lesions). These may occur on the end of the penis (glans penis), the undersides of the hands (palmar surfaces), soles of the feet, and in the mouth. Lesions may also develop in the bladder and the urinary tubes from the bladder (urethra). These lesions may become eroded and red. However, they usually cause little pain and resolve quickly.

Some skins lesions of Reiter's Syndrome, associated with Keratoderma Blennorrhagica, are found mostly on the hands, arms, and trunk of the body. They are scaly, crusty, and eventually peel off. These rashes are similar to those of pustular psoriasis. The fingernails may also become brittle, thick, and/or opaque. (For more information on this disorder, choose "Psoriasis" as your search term in the Rare Disease Database.)

In rare cases, some people with Reiter's Syndrome develop abnormalities of the heart. These may include impaired function of the valves of the heart (aortic valve) and abnormalities in the heart's ability to contract normally (cardiac conduction defects).

Reiter's Syndrome occurs because of an infection which may be sexually acquired (Chlamydia trachomatis or Ureaplasma urealyticum). Other nonsexually transmitted bacterial organisms that may cause Reiter's Syndrome include Shigella flexneri, Salmonella, Yersinia, and Campylobacter jejuni.

Research suggests that approximately 63 to 96 percent of individuals with Reiter's Syndrome have a genetic predisposition (HLA-B27) to this disorder. The presence of HLA-B27 antigen on chromosome 6 is a predisposing factor to this syndrome. A genetic predisposition means that a person may carry a gene for a disease but it may not be expressed unless something in the environment triggers the disease.

Recently, Reiter's syndrome has been diagnosed in men with symptomatic HIV infection and AIDS. This is probably because of their increased risk of exposure to the above organisms.

Reiter's Syndrome is a rare disorder that occurs almost exclusively in males between 20 to 40 years of age. It is most frequently acquired through sexual contact (venereal), although it may occur because of infections that are not sexually transmitted (enteric). The HLA-B27 antigen, which is associated with a genetic predisposition to this syndrome, is present in 6 to 14 percent of Americans of European descent and in 1 to 4 percent of Americans of African descent. Reiter's Syndrome may also occur in women, children, and the elderly. It is often undiagnosed or misdiagnosed for an extended period of time.

Symptoms of the following disorders can be similar to those of Reiter's Syndrome. Comparisons may be useful for a differential diagnosis:

Many cases of Reiter Syndrome can be prevented with the use of barrier contraceptives which prevent transmission of venereal diseases.

Laboratory testing of tissue samples taken from the urogenital tracts of people with Reiter's Syndrome may be positive for Chlamydia, a sexually transmitted bacteria. Gonorrhea must be ruled out through other tests. Other organisms (i.e., Shigella flexneri) that may be present in the bowel (enteric infection), may also be cultured in stool samples. Laboratory blood tests may also reveal low levels of hemoglobin (anemia), abnormally high levels of white blood cells in the blood and the fluid that surrounds the joints (synovial), and an elevated sedimentation rate. Tests for rheumatoid factor are negative (sera-negative arthropathy).

The treatment of Reiter's Syndrome is symptomatic and supportive. Nonsteroidal antiinflammatory drugs (NSAIDs) such as indomethacin, diclofenac, tolmetin, sulindac, or phenylbutazone usually provide relief of the symptoms associated with arthritis. In severe cases, folic acid antagonists such as methotrexate may relieve joint inflammation. Methotrexate, which acts as an immunosuppressant, must be used with extreme caution because of the potential harmful side effects.

Physical therapy may be useful during recovery from arthritis associated with Reiter's Syndrome. Inflammation of the urinary tract can be treated with the antibiotic tetracycline. Other antibiotics may be prescribed to treat infections of the bowel.

People with Reiter's Syndrome who have severe, recurring inflammation that effects the eyes, may require eye drops that contain steroids to help control inflammation.

The use of sulfasalazine is being tested for the treatment of seronegative spondyloarthropathies. The drug has been found to be helpful in the treatment of Psoriatic Arthritis, Infectious Arthritis, Reiter's Syndrome, and Akylosing Spondylitis with peripheral joint involvement. More studies are needed to determine the long-term safety and effectiveness of this treatment.

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

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