$Unique_ID{BRK03472} $Pretitle{} $Title{Ankylosing Spondylitis} $Subject{Ankylosing Spondylitis Marie Strumpell disease Von Bechterew-Strumpell syndrome Spondyloarthritis Rheumatoid Arthritis Reiter Syndrome Psoriatic Arthritis Enteropathic Arthritis} $Volume{} $Log{} Copyright (C) 1986, 1987, 1988, 1991 National Organization for Rare Disorders, Inc. 143: Ankylosing Spondylitis ** IMPORTANT ** It is possible the main title of the article (Ankylosing Spondylitis) is not the name you expected. Please check the SYNONYMS listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Marie Strumpell disease Von Bechterew-Strumpell syndrome Spondyloarthritis Information on the following diseases can be found in the Related Disorders section of this report: Rheumatoid Arthritis Reiter Syndrome Psoriatic Arthritis Enteropathic Arthritis General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section. Ankylosing Spondylitis is a chronic progressive form of arthritis distinguished by inflammation and eventual immobility (ankylosis) of a number of joints. It primarily involves the spine and paraspinal structures. Symptoms However, these symptoms can appear as the first symptom in twenty percent of patients. Onset of Ankylosing Spondylitis is usually gradual with episodes of low back pain, especially in the sacroiliac (tailbone and hips) and lumbar regions. Pain may occur in the area innervated by the sciatic nerve, i.e., radiating from the back into the buttock and down a lower extremity. Well- defined morning back stiffness often occurs. Symptoms commonly become progressively worse, spreading from the low back frequently into the mid back and occasionally the neck. Peripheral joints such as the hips and shoulders may be affected at any stage of the disease. However, these symptoms can appear as the first symptom in twenty percent of patients. Involvement of the heart occurs in less than 10% of cases, including cardiac arrhythmias or aortic insufficiency, after longstanding disease. Sacroiliac joint involvement usually is an early sign on X-rays. Symptoms initially may be limited to tenderness of those joints or of the low back. The normal lumbar concave curve may be flattened because of muscle spasm and involvement of adjacent spinal joints. Normal flexion and extension of the spine does not occur during bending of the back. This is due to muscle spasms early in the course of the disease and to bony immobility (ankylosis) later. The joints between ribs and vertebrae may also be involved causing diminution of chest expansion and a decrease in the maximum amount of air that can be exhaled (vital capacity). Neck movements may be mildly or seriously limited. Immobility of the entire spine, resulting in an erect "poker spine", or a hump back (dorsal kyphosis), may develop from bending forward of the chest portion of the spine in advanced Ankylosing Spondylitis. Causes The cause of Ankylosing Spondylitis is unknown. However, researchers have discovered two genetic markers which may signal a susceptibility for development of this disorder. Although these markers appear to be inherited independently of each other, tests show that an individual with both of them has almost three hundred times greater risk of developing Ankylosing Spondylitis than the person who has neither. Most of the evidence suggests that the disease occurs when an individual bearing a predisposing gene is exposed to an as yet unidentified environmental factor such as bacteria or a virus. Affected Population Ankylosing Spondylitis is found among caucasian males approximately three times as often as in females, and usually begins between the ages of sixteen and thirty-five years. Symptoms of Ankylosing Spondylitis are generally milder among females. This disorder rarely occurs in the black population. Approximately 2.5 million Americans may have this disease. Related Disorders Rheumatoid Arthritis is a chronic syndrome characterized by usually symmetric inflammation of the peripheral joints, which eventually can potentially result in progressive destruction of bone joints. (For more information on Rheumatoid Arthritis, please see articles in the Prevalent Health Conditions/Concerns area of NORD Services and choose "arthritis" as your search term in the Rare Disease Database.) Reiter Syndrome is characterized by inflammation of the joints, urethra, and conjunctiva of the eye, and by lesions of the skin and mucosal surfaces. Symptoms do not necessarily appear simultaneously; they may alternate, and there may be spontaneous remissions and recurrences. The syndrome rarely disables its victims. It appears to result from an abnormal immune response in association with exposure (usually sexual) to an infectious agent. It usually affects men between the ages of twenty and forty years. (For more information on this disorder, choose "Reiter" as your search term in the Rare Disease Database.) Psoriatic Arthritis is a rheumatoid-like arthritic condition which is associated with psoriasis of the skin or nails, and a negative rheumatoid arthritis (RA) serology laboratory test. The disorder is more common among females than males. (For more information on this disorder, choose "Psoriatic Arthritis" as your search term in the Rare Disease Database.) Enteropathic Arthritis is a form of arthritis sometimes resembling rheumatoid arthritis which may complicate the course of ulcerative colitis or Crohn's disease. This disorder often develops early in adult life as a peripheral arthritis which affects many patients with ulcerative colitis and fewer of those with regional enteritis (Crohn's disease). A peripheral type of arthritis begins abruptly, peaking within twelve to twenty-four hours, and usually involves six to eight joints. Attacks may last weeks or months and can become worse as bowel disease becomes more severe. A spondylitic form of arthritis begins gradually and without warning. Sacroiliac joints are most often affected. The hips and shoulders are affected less often, and eye problems may also occur. (For more information, Choose "Crohn" and "Ulcerative Colitis" as your search terms in the Rare Disease Database.) Therapies: Standard Posture-maintaining exercises should be performed as soon as the diagnosis of Ankylosing Spondylitis is made, to retain as much normal upright posture as possible. Exercises should stress back movements, straightening of the chest portion of the spine, deep-bending exercises and as full a range of motion of the spine in all directions as possible. Bending (flexion) postures should not be maintained for long periods of time. To avoid flexion of the neck and upper back, the patient should sleep on his back on a firm mattress and use only a small pillow or none at all. The chest muscles should be stretched and the upper back straightened by locking the fingers behind the head and pushing the elbows as far back as possible. Hot baths or warm showers are helpful to relax muscles and are used more frequently than hot packs before exercising to attain better range of motion. The patient should have ample rest each day and avoid exhaustion. Analgesics (painkillers) may help relieve pain, permitting better sleep and increasing the ability to exercise. Nonsteroidal anti-inflammatory drugs are of positive benefit to many patients with Ankylosing Spondylitis. Aspirin can be less satisfactory than others, but may be helpful in some patients. Narcotics and systemic corticosteroids should be avoided. X-ray therapy to the back is not recommended (contraindicated). If the above measures are followed, surgical procedures to straighten the spine will only rarely be needed. Some patients may benefit from a back brace, but it is often unnecessary. Therapies: Investigational The Department of Health and Human Services released information in November of 1990 that a protein molecule, HLA-B27, known to be a genetic marker for Ankylosing Spondylitis and some forms of Arthritis, has aided researchers in developing an animal model for the disease. This will be a great help in understanding and treating patients suffering from Arthritis and Ankylosing Spondylitis. This disease entry is based upon medical information available through February 1991. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Ankylosing Spondylitis, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 Ankylosing Spondylitis Association 511 North La Cienega, Suite 216 Los Angeles, CA 90048 (213) 652-0609 (800) 777-8189 Arthritis Foundation 1314 Spring Street Atlanta, GA 30309 (404) 872-7100 The National Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse Box AMS Bethesda, MD 20892 (301) 495-4484 References CECIL TEXTBOOK OF MEDICINE, 18th ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Eds.: W. B. Saunders Co., 1988. Pp. 2006-7, 1968. THE MERCK MANUAL 15th ed: R. Berkow, et al: eds; Merck, Sharp & Dohme Research Laboratories, 1987. P. 1247.