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Article title: Questions and Answers About Reiter's Syndrome: NIAMS
Publication Date: April 1999
Questions and Answers About Reiter's Syndrome
This fact sheet contains general information about Reiterís syndrome. It describes what Reiterís syndrome is and how it develops. It also explains how Reiterís syndrome is diagnosed and treated. If you have further questions after reading this fact sheet, you may wish to discuss them with your doctor.
- What Is Reiterís Syndrome?
- What Causes Reiterís Syndrome?
- Is Reiterís Syndrome Contagious?
- Who Gets Reiterís Syndrome?
- What Are the Symptoms of Reiterís Syndrome?
- How Is Reiterís Syndrome Diagnosed?
- What Type of Doctor Treats Reiterís Syndrome?
- How Is Reiterís Syndrome Treated?
- What Is the Prognosis for People Who Have Reiterís Syndrome?
- What Are Researchers Trying To Learn About Reiterís Syndrome?
- Where Can People Get More Information About Reiterís Syndrome?
- Key Words
Reiterís syndrome is a disorder that causes three seemingly unrelated symptoms: arthritis, redness of the eyes, and urinary tract signs. Doctors sometimes refer to Reiterís syndrome as a seronegative spondyloarthropathy because it is one of a group of disorders that cause inflammation throughout the body, particularly in parts of the spine and at other joints where tendons attach to bones. (Examples of other seronegative spondyloarthropathies include psoriatic arthritis, ankylosing spondylitis, and inflammatory bowel syndrome arthritis.) Inflammation is a characteristic reaction of tissues to injury or disease and is marked by four signs: swelling, redness, heat, and pain.
Reiterís syndrome is also referred to as reactive arthritis, which means that the arthritis occurs as a ďreactionĒ to an infection that started elsewhere in the body. In many patients, the infection begins in the genitourinary tract (bladder, urethra, penis, or vagina). The infection is most commonly passed from one person to another by sexual intercourse. This form of the disorder is sometimes called genitourinary or urogenital Reiterís syndrome. Another form of the disorder, called enteric or gastrointestinal Reiterís syndrome, develops when a person eats food or handles substances that are tainted with bacteria.
When a preceding infection is recognized, symptoms of Reiterís syndrome appear about 1 to 3 weeks after the infection. Chlamydia trachomatis is the bacteria most often associated with Reiterís syndrome acquired through sexual contact. Several different bacteria are associated with Reiterís syndrome acquired through the digestive tract, including Salmonella, Shigella, Yersinia, and Campylobacter. People may become infected with these bacteria after eating or handling improperly prepared food, such as meats that are not stored at the correct temperature.
Doctors do not know exactly why some people exposed to these bacteria develop the disorder and others do not, but they have identified a genetic factor (HLAĖB27) that increases a personís chance of developing Reiterís syndrome. About 80 percent of people with Reiterís syndrome are HLAĖB27 positive. Only 6 percent of people who do not have the syndrome have the HLAĖB27 gene.
Reiterís syndrome is not contagious; that is, a person with the disorder cannot pass it to somebody else. However, the bacteria that can trigger it can be passed from one person to another, although not all people infected with the bacteria will develop Reiterís syndrome. Rather, it is likely that people who develop the disease have inherited a trait that makes them susceptible.
Men between the ages of 20 and 40 are most likely to develop Reiterís syndrome. It is the most common type of arthritis affecting young men. Among men under age 50, about 3.5 per 100,000 develop Reiterís syndrome each year. Three percent of all men with a sexually transmitted disease develop Reiterís syndrome. Women can also develop the disorder, though less often than men, with features that are often milder and more subtle.
The symptoms can affect many different parts of the body, but most typically affect the urogenital tract, the joints, and the eyes. Less common symptoms are mouth ulcers, skin rashes, and heart-valve problems. The signs may be so mild that patients do not notice them. They usually come and go over a period of several weeks to several months.
Urogenital Tract Symptoms
Reiterís syndrome often affects the urogenital tract, including the prostate, urethra, and penis in men and the fallopian tubes, uterus, and vagina in women. Men may notice an increased need to urinate, a burning sensation when urinating, and a discharge from the penis. Some men with Reiterís syndrome develop prostatitis, inflammation of the prostate gland. Symptoms of prostatitis can include fever, chills, increased need to urinate, and a burning sensation when urinating.
Women with Reiterís syndrome also develop signs in the urogenital tract, such as inflammation of the cervix (cervicitis) or inflammation of the urethra (urethritis), which can cause a burning sensation during urination. In addition, some women also develop salpingitis (inflammation of the fallopian tubes) or vulvovaginitis (inflammation of the vulva and vagina). These conditions may or may not cause any symptoms.
Joint Symptoms or Arthritis
The arthritis associated with Reiterís syndrome typically affects the knees, ankles, and feet, causing pain and swelling. Wrists, fingers, and other joints are less often affected. Patients with Reiterís syndrome commonly develop inflammation where the tendon attaches to the bone, a condition called enthesopathy. Enthesopathy may result in heel pain and the shortening and thickening of fingers and toes. Some people with Reiterís syndrome also develop heel spurs, bony growths in the heel that cause chronic or long-lasting foot pain.
Arthritis in Reiterís syndrome can also affect the joints in the back and cause spondylitis (inflammation of the vertebrae in the spinal column) or sacroiliitis (sa-kro-il-e-i-tes), inflammation of the joints in the lower back that connect the spine to the pelvis. People with Reiterís syndrome who have the HLAĖB27 gene have a greater chance of developing sacroiliitis and spondylitis.
Conjunctivitis, an inflammation of the mucous membrane that covers the eyeball and eyelid, develops in about 50 percent of people with urogenital Reiterís syndrome and 75 percent of people with enteric Reiterís syndrome. A few people may develop uveitis, an inflammation of the inner eye. Conjunctivitis and uveitis can cause redness of the eyes, eye pain and irritation, and blurred vision. Eye involvement typically occurs early in the course of Reiterís syndrome, and symptoms may come and go.
About 20 to 40 percent of men with Reiterís syndrome develop small, shallow, painless sores or lesions, called balanitis circinata, on the end of the penis. A small percentage of men and women develop rashes of small hard nodules on the soles of the feet, and less often on the palms of the hands or elsewhere. These rashes are called keratoderma blennorrhagica. In addition, some people with Reiterís syndrome develop mouth ulcers that come and go. In some cases, these ulcers are painless and go unnoticed.
About 10 percent of people with Reiterís syndrome, usually those with prolonged disease, develop heart problems including aortic regurgitation (leakage of blood from the aorta into the heart chamber) and pericarditis (inflammation of the membrane that covers and protects the heart).
Diagnosing Reiterís syndrome is often difficult because there is no specific test to confirm that a person has it. When a patient reports symptoms, the doctor must examine him or her carefully and rule out other causes of arthritis.
The doctor will take the patientís complete medical history, noting current symptoms as well as any previous diseases, problems, and infections. Because the symptoms of Reiterís syndrome can be vague, it is sometimes useful for the patient to keep a log of the symptoms that occur, when they occur, and for how long. It is especially important to report any flulike symptoms, such as fever, vomiting, or diarrhea, even if they were mild, because they may be associated with the initial bacterial infection.
The doctor may use various blood tests to help rule out other conditions and confirm a suspected diagnosis of Reiterís syndrome. Tests may be done to determine the presence of rheumatoid factor or antinuclear antibodies. Results of these tests are abnormal in patients with other types of arthritis such as rheumatoid arthritis or lupus, but they typically are normal in patients with Reiterís syndrome. Doctors may determine the erythrocyte sedimentation rate, or sed rate, which is the rate at which red blood cells settle at the bottom of a test tube of blood. An elevated sed rate indicates inflammation somewhere in the body. Typically, people with rheumatic diseases, including Reiterís syndrome, have an elevated sed rate. In some patients with suspected Reiterís syndrome, the doctor may do a blood test to determine the presence or absence of HLAĖB27.
The doctor is also likely to perform tests for infections that might be associated with Reiterís syndrome. Patients are generally tested for a Chlamydia infection because recent studies have shown that early treatment in Chlamydia-induced Reiterís syndrome may ameliorate the course of the disease. In many people with Reiterís syndrome, there is no clear evidence of infection at the time they are seen, although antibodies may be detected in the blood, indicating that an infection was present in the past. The doctor may test samples of cells taken from the patientís throat as well as the urethra in men or cervix in women. Urine and stool samples may also be tested. The synovial fluid (the fluid that lubricates the joints) or the membrane (synovium) that lines the joint may be removed from the joint affected by arthritis. Studies of the fluid or the synovium can help the doctor make certain there is no infection in the joint.
Doctors sometimes use X rays to help establish a diagnosis of Reiterís syndrome and rule out other causes of arthritis. Common findings on X rays of patients with Reiterís syndrome include spondylitis, sacroiliitis, swelling of soft tissues, damage to cartilage or bone margins of the joint, and bone deposits where the tendon attaches to the bone.
A patient probably will see different doctors because Reiterís syndrome affects different parts of the body. It may be helpful to the doctors and the patient for one doctor to manage the complete treatment plan. This doctor can coordinate treatments and monitor the side effects from the various medicines the patient may take. A rheumatologist (doctor specializing in arthritis) often manages a patientís treatment and treats the joint disease. The following specialists treat other features that affect different parts of the body.
Ophthalmologistótreats eye disease.
Gynecologistótreats urogenital symptoms in women.
Urologistótreats urogenital symptoms in men.
Dermatologistótreats skin symptoms.
Orthopaedistóperforms surgery on severely damaged joints.
Physiatristósupervises exercise regimens.
Although there is no cure for Reiterís syndrome, treatments that effectively relieve the symptoms are available. Many symptoms may even disappear for long periods of time. The doctor is likely to use one or more of the following treatments:
Bed restóShort periods of bed rest are sometimes effective in reducing the pain and inflammation of arthritis. Lying down can reduce the pressure of the bodyís weight on a painful joint and provide relief for some patients.
ExerciseóEven before symptoms disappear, some strengthening and gentle range-of-motion exercises will maintain or improve joint function. Strengthening exercises build up the muscles around the joint to better support it. Isometric tightening of muscles without moving the joints can be used even in active, painful disease. Range-of-motion exercises improve movement and flexibility and reduce stiffness in the affected joint. Before beginning an exercise program, patients should talk to the doctor, who can recommend appropriate exercises.
Nonsteroidal anti-inflammatory drugs (NSAIDís)óThis type of medicine effectively reduces joint inflammation and is commonly used to treat patients with Reiterís syndrome. Some NSAIDís, such as aspirin and ibuprofen, are available without a prescription. Many others require a doctorís prescription.
Corticosteroid injectionsóFor people with severe joint inflammation, injections of corticosteroids directly into the affected joint may effectively reduce inflammation. Doctors typically use this treatment only after trying to control arthritis with NSAIDís. Corticosteroid injections are most commonly used for severe knee or ankle inflammation.
Topical corticosteroidsóThis type of medicine can be put directly on the skin lesions associated with Reiterís syndrome. Topical corticosteroids reduce inflammation and promote healing.
AntibioticsóAntibiotics may be prescribed to eliminate the bacterial infection that triggered Reiterís syndrome. The specific antibiotic prescribed depends on the type of bacterial infection that has to be treated. Patients must carefully follow the doctorís instructions about how much medicine to take and for how long; if the medicine is not taken correctly, the infection may not go away. Often, an antibiotic is taken once or twice a day for 7 to 10 days or longer. Some doctors may recommend that a person with Reiterís syndrome take antibiotics for a long period of time (up to 3 months). Current research shows that this practice usually has no effect on the course of the disease and is therefore unnecessary. However, in cases when Chlamydia triggers Reiterís syndrome, prolonged antibiotic treatment is effective in shortening the length of time that a person has symptoms.
Immunosuppressive medicinesóA small percentage of patients with Reiterís syndrome have severe symptoms that cannot be controlled with the treatments described earlier. For these people, medicine that suppresses the immune system, such as sulfasalazine or methotrexate, may be effective.
Most people with Reiterís syndrome recover fully from the initial flare of symptoms and are able to return to regular activities within 2 to 6 months after the first symptoms appear. Arthritis may last up to 6 months, although the symptoms are usually very mild and do not interfere with daily activities. Only 20 percent of people with Reiterís syndrome will have chronic arthritis, which is usually mild. Some patients experience symptom recurrence. Studies show that about 15 to 50 percent of patients will develop symptoms sometime after the initial flare has disappeared. Back pain and arthritis are the symptoms that most commonly reappear. A small percentage of patients will have deforming arthritis and severe symptoms that are difficult to control with treatment.
Researchers continue to investigate the causes of Reiterís syndrome and study treatments for the condition. For example:
Researchers are trying to better understand the relationship of infection to Reiterís syndrome. In particular, they are trying to determine why an infection triggers arthritis and why some people who develop infections get Reiterís syndrome and others do not. Scientists have identified a genetic linkópeople who are positive for HLAĖB27 are more susceptible to Reiterísóand are studying why these people are more at risk than others.
Researchers are trying to develop methods to detect the location of the triggering bacteria in the body. Some scientists suspect that after the bacteria enter the body, they are transported to the joints, where they can remain in small amounts indefinitely.
Researchers are studying new treatments for Reiterís syndrome; for example, prolonged treatment with antibiotics or a combination of antibiotics and other drugs such as methotrexate or sulfasalazine. Several recent studies have shown that prolonged treatment with antibiotics may reduce the duration of symptoms in some patients with Reiterís syndrome caused by Chlamydia infection.
Spondylitis Association of America
P.O. Box 5872
Sherman Oaks, CA 91413
World Wide Web address: http://www.spondylitis.org/
This is the main voluntary organization devoted to all forms of spondylitis, including Reiterís syndrome. The association publishes patient and professional materials and a newsletter for members.
1330 West Peachtree Street
Atlanta, GA 30309
World Wide Web address: http://www.arthritis.org/
This is the main voluntary organization devoted to arthritis. The foundation publishes a free pamphlet on Reiterís syndrome and can also provide physician/clinic referrals.
American College of Rheumatology
60 Executive Park South, Suite 150
Atlanta, GA 30329
World Wide Web address: http://www.rheumatology.org/
This professional organization of rheumatologists, both physicians and scientists, is dedicated to treating and studying all forms of arthritis and can also provide a list of rheumatologists by State.
National Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse (NAMSIC)
National Institutes of Health
1 AMS Circle
Bethesda, MD 20892Ė3675
World Wide Web address: http://www.niams.nih.gov/index.htm
This clearinghouse, a public service sponsored by the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), provides information about various forms of arthritis and rheumatic disease. The clearinghouse distributes patient and professional education materials and also refers people to other sources of information.
The NIAMS gratefully acknowledges the assistance of John Klippel, M.D., of NIAMS; Daniel Clegg, M.D., University of Utah School of Medicine, Salt Lake City; and Ralph Schumacher, M.D., VA Medical Center, Philadelphia, PA, in the preparation and review of this fact sheet.
The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), a part of the National Institutes of Health (NIH), leads the Federal medical research effort in arthritis and musculoskeletal and skin diseases. The NIAMS supports research and research training throughout the United States, as well as on the NIH campus in Bethesda, MD, and disseminates health and research information. The National Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse (NAMSIC) is a public service sponsored by the NIAMS that provides health information and information sources. Additional information can be found on the NIAMS Web site at http://www.niams.nih.gov/index.htm.
Key Words Antibodies: Special proteins produced by the bodyís immune system that recognize and help fight infectious agents, such as bacteria, and other foreign substances that invade the body. Antinuclear antibodies: Abnormal antibodies that are often present in people who have connective tissue diseases or other autoimmune disorders. Arthritis: Literally means joint inflammation. It is a general term for more than 100 conditions known as rheumatic diseases. These diseases affect not only the joints but also other parts of the body, including important supporting structures such as muscles, tendons, and ligaments, as well as some internal organs. Bacteria: Any group of single-celled micro-organisms that live in soil, water, and organic matter or in the bodies of plants, animals, and humans. Some types of bacteria cause illness when they enter the body. Balanitis circinata: Small, shallow, painless sore on the penis. Conjunctivitis: Inflammation of the mucous membrane that covers the eyeball and eyelid. Corticosteroids: Potent anti-inflammatory hormones that are made naturally in the body or synthetically (man-made) for use as drugs. They are also called glucocorticoids. The most commonly prescribed drug of this type is prednisone. Enteric: A term related to the intestines and the digestion of food. Enthesopathy: Inflammation where the tendon attaches to the bone. This symptom is unique to the seronegative spondyloarthropathies. Erythrocyte sedimentation rate: A blood test that measures the speed at which red blood cells settle sedimentation at the bottom of a test tube. A high rate signals possible inflammatory disease. Also referred to as the ďsedĒ rate or ďESR.Ē Gastrointestinal tract: Organs related to the digestion of food, including the stomach and the intestines. HLAĖB27: A genetic marker that may be found in the blood of patients with certain forms of arthritis such as ankylosing spondylitis and Reiterís syndrome. Immuno-suppressive drugs: Medicines that decrease the immune response and may relieve some symptoms of severe Reiterís syndrome. Inflammation: A characteristic reaction of tissues to injury or disease. It is marked by four signs: swelling, redness, heat, and pain. Keratoderma blennorrhagica: Red patches that usually appear on the bottom of the foot. The area may look like excessively dry skin. NSAID: An abbreviation for nonsteroidal anti-inflammatory drug. NSAIDís do not contain corticosteroids and are used to reduce pain and inflammation. Aspirin and ibuprofen are two types of NSAIDís, but there are many others. Range of motion: A measurement of the extent to which a joint can go through all of its normal movements. Reactive arthritis: A systemic illness caused by an infection. The most common symptom is joint inflammation. Rheumatoid arthritis: A chronic inflammatory disease that causes pain, stiffness, swelling, and loss of function in the joints. The primary target of rheumatoid arthritis is the synovium, or joint lining. This tissue, which normally is smooth and shiny, becomes inflamed, painful, and swollen. The disease can also cause inflammation in the blood vessels and the outer lining of the heart and lungs. Rheumatoid factor: A special kind of antibody often found in people with some types of rheumatic diseases, but usually not Reiterís syndrome. Sacroiliitis: Inflammation of the sacrum. Sacrum: The part of the spine that connects it to the pelvis. Seronegative spondylo- arthropathy: A category of diseases with several similarities, including the presence of HLAĖB27 in the blood, enthesopathy, sacroiliitis, and systemic symptoms. Examples include Reiterís syndrome, psoriatic arthritis, ankylosing spondylitis, and inflammatory bowel syndrome arthritis. Spondylitis: Inflammation of the vertebrae. Synovial fluid: The fluid that lubricates the joints and reduces friction during movement. Urethritis: Inflammation of the urethra, the canal that carries urine away from the bladder and, in males, also carries semen. Urogenital tract: Organs related to the production and excretion of urine and to reproduction. Uveitis: Inflammation of the inner eye, which includes the iris, the ciliary body that holds the lens of the eye; and the choroid plexus, a network of blood vessels surrounding the eyeball.
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