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$Unique_ID{BRK04122}
$Pretitle{}
$Title{Polymyositis}
$Subject{Polymyositis Primary Idiopathic Polymyositis Childhood Polymyositis
Polymyositis malignant tumors Polymyositis connective tissue disease overlap
syndromes Sclerodermatomyositis Mixed Connective Tissue disease}
$Volume{}
$Log{}
Copyright (C) 1986, 1989, 1991, 1992 National Organization for Rare
Disorders, Inc.
278:
Polymyositis
** IMPORTANT **
It is possible the main title of the article (Polymyositis) is not the
name you expected. Please check the SYNONYMS listing to find the alternate
names and disorder subdivisions covered by this article.
Synonyms
DISORDER SUBDIVISIONS
Primary Idiopathic Polymyositis
Childhood Polymyositis
Polymyositis associated with malignant tumors
Polymyositis associated with connective tissue disease overlap syndromes,
including Sclerodermatomyositis and Mixed Connective Tissue disease
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 of this report.
Polymyositis is a systemic connective tissue disorder characterized by
inflammatory and degenerative changes in the muscles, leading to symmetric
weakness and some degree of muscle atrophy. The areas principally affected
are the hip, shoulders, arms, pharynx and neck.
Symptoms
Symptoms of Polymyositis may start gradually or suddenly. The symptoms often
wax and wane for no apparent reason.
The major symptom of the disorder is muscle weakness, most often in the
hip and shoulder areas, eventually making it difficult for patients to lift
their arms or to climb steps. Other muscles which may be affected are the
neck and throat muscles, which may result in difficulty in swallowing and
cause changes in the voice. Rarely, chest muscles are affected.
The muscle weakness may appear suddenly and progress over weeks to
months. The difficulty in swallowing and dilatation of the lower esophagus
and small intestine may be indistinguishable from that in Scleroderma
(Progressive Systemic Sclerosis), (For more information on Scleroderma,
choose "scleroderma" as your search term in the Rare Disease Database.) The
muscles of the hands, feet and face often escape involvement. Contractures
of the limbs may develop late in the chronic stage.
Other symptoms of Polymyositis may include fever, weight loss and
occasionally pain or tenderness in muscles and joints. A few people with
Polymyositis have an extreme sensitivity to cold (Raynaud's Phenomenon) that
is most often felt in the fingers. Raynaud's Phenomenon is caused by
narrowing of the blood vessels in the fingers. (For more information, choose
"Raynaud" as your search term in the Rare Disease Database.) People with
Polymyositis may develop numb and shiny red areas around and under the finger
nails.
Pain in many joints (polyarthralgia), accompanied at times by swelling,
fluid and other evidence of non-deforming arthritis, occurs in approximately
one third of patients with polymyositis. These rheumatic complaints tend to
be mild and respond well to corticosteroids. Gastrointestinal involvement,
except for the pharynx and the esophagus, is relatively uncommon in
polymyositis.
Inflammation of the lungs with increase of interstitial tissue (interstitial
pneumonitis), manifested by difficulty in breathing and by coughing, may
precede myositis and dominate the clinical picture. Involvement of the heart,
detected chiefly by irregularities in the electrocardiogram (ECG), has been
reported. Acute kidney failure has been reported as a consequence of excess
muscle protein myoglobin in the urine (Crush syndrome) due to severe
disintegration of muscle (rhabdomyolysis). Sjogren's syndrome can occur in
some patients with polymyositis. (For more information, choose "Sjogren" as
your search term in the Rare Disease Database.) Abdominal symptoms, more
common in children, may be associated with the passage of dark stools or the
vomiting of blood from gastro-intestinal ulcerations that may progress to
perforation and require surgical intervention.
An associated malignancy, usually a carcinoma, may occur in about 15% of
men and a smaller proportion of women over age 50 with polymyositis.
Causes
The cause of polymyositis is unknown. The disorders may be caused by the
body's natural immune defense mechanisms attacking its own tissue (autoimmune
reaction). Viruses may also play a role.
Affected Population
Polymyositis may appear at any time from infancy through the age of 80 years,
but most commonly they occur between 40 to 60 years. In children, the
symptoms usually appear between the ages of 5 to 15 years. Females are
affected twice as often as males.
Related Disorders
Scleroderma (Progressive Systemic Sclerosis) is a rare, chronic collagen
vascular disorder characterized by diffuse hardening, degenerative changes
and vascular inflammation of the connective tissues of the skin, joints and
many visceral organs. It shares certain clinical findings with polymyositis.
Systemic Lupus Erythematosus (SLE) is an inflammatory connective tissue
disorder that can affect many parts of the body including the joints, skin
and internal organs. SLE is a disease of the body's immune system. It
shares certain clinical findings with Polymyositis.
(For more information on these related disorders, choose "Scleroderma"
and "Lupus" as your search terms in the Rare Disease Database.)
Therapies: Standard
Corticosteroids such as prednisone, (together with antacids and potassium
supplements), are widely used in treatment of Polymyositis. Measurement of
muscle enzyme activity is used to gauge the effectiveness of therapy.
Reduction of these enzymes to normal values is noted in a majority of
patients with this disorder within 4 to 6 weeks after treatment is started.
This is followed by an improvement in muscle strength. At this point the
dose of prednisone can usually be reduced slowly. In many cases of adult
polymyositis prolonged maintenance therapy with prednisone may be necessary
indefinitely.
Immunosuppressive drugs such as methotrexate, cyclophosphamide,
chlorambucil and azathioprine have been beneficial to patients who fail to
respond to corticosteroids alone. Some patients have received methotrexate
for 5 years or longer for control of this disorder.
Therapies: Investigational
The FDA has approved the following orphan product for treatment of
Polymyositis:
Immune Globulin Intravenous (Human) (Iveegam, Immuno)
Sponsored by:
Immuno Clinic Research Corp.
155 E. 56th St.
New York, NY 10022
The drug cyclophosphamide, in combination with the drug mesna, is being
tested in severe polymyositis patients who are unresponsive to steroid
immunosuppressant therapy. This therapy may be beneficial, but more research
is needed to determine the long-term safety and effectiveness.
This disease entry is based upon medical information available through
November 1992. 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 Polymyositis, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
Dermatomyositis and Polymyositis Support Group
146 Newtown Rd.
Woolston, Southhampton
SO2 9HR England
Phone Southhampton 449708
The National Arthritis and Musculoskeletal and Skin Diseases Information
Clearinghouse
Box AMS
Bethesda, MD 20892
(301) 495-4484
Arthritis Foundation
1314 Spring St., NW
Atlanta, GA 30333
(404) 872-7100
References
POLYMYOSITIS AND DERMATOMYOSITIS: C.M. Pearson; Arthritis Medical Information
Series, Arthritis Foundation, 1983.