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$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
     [phone]

     Ankylosing Spondylitis Association
     511 North La Cienega, Suite 216
     Los Angeles, CA  90048
     [phone]
     [phone]

     Arthritis Foundation
     1314 Spring Street
     Atlanta, GA 30309
     [phone]

     The National Arthritis and Musculoskeletal and
     Skin Diseases Information Clearinghouse
     Box AMS
     Bethesda, MD  20892
     [phone]

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.