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$Unique_ID{BRK04034}
$Pretitle{}
$Title{Myositis, Inclusion Body}
$Subject{Myositis, Inclusion Body IBM Inflammatory Myopathy Dermatomyositis
Idiopathic Polymyositis Distal Myopathy Mixed Connective Tissue Disease
Oculopharyngeal Muscular Dystrophy }
$Volume{}
$Log{}
Copyright (C) 1989 National Organization for Rare Disorders, Inc.
649:
Myositis, Inclusion Body
** IMPORTANT **
It is possible that the main title of the article (Inclusion Body
Myositis) is not the name you expected. Please check the SYNONYM listing to
find the alternate names and disorder subdivisions covered by this article.
Synonyms
IBM
Disorder Subdivisions:
Inflammatory Myopathy
Information on the following diseases can be found in the Related
Disorders section of this report:
Dermatomyositis
Idiopathic Polymyositis
Distal Myopathy
Mixed Connective Tissue Disease
Oculopharyngeal Muscular Dystrophy
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.
Inclusion Body Myositis (IBM) is a slowly progressive weakness of muscle
groups located either away from the point of origin (distal) or nearest to
the trunk or point of origin (proximal).
Symptoms
Inclusion Body Myositis (IBM) is characterized by a distinct, progressive
muscle weakness of the proximal and distal muscles. It is recognized as an
inflammatory disease of the skeletal muscles. IBM is not usually associated
with skin rash, malignancy, or collagen vascular disease. However,
exceptions to each of these general rules has been found in certain patients.
Clinically IBM differs from dermatomyositis and polymyositis because it lacks
the features of a collagen vascular disease which include symptoms of fever,
headache, joint and muscle pain, and weakness. It has a relatively benign
and protracted course.
In polymyositis and Inclusion Body Myositis there is evidence of
macrophages (scavenger cells) which surround, invade, and destroy non-
necrotic (living) muscle fibers.
Disorder Subdivisions:
Inflammatory Myopathy is an inflammation of the muscle groups, which
causes progressive muscle weakness.
Causes
Inclusion Body Myositis seems to be a distinct type of inflammatory muscle
disease. It's cause is unknown. A virus or possibly a neurogenic cause
(related to or starting in the nervous system) has been suggested, but both
theories remain unproven.
Affected Population
IBM occurs primarily in elderly persons with the average age of onset being
53. However, young adults in their teens have been affected. It seems to
affect mostly males.
Related Disorders
Symptoms of the following disorders can be similar to those of Inclusion Body
Myositis. Comparisons may be useful for a differential diagnosis:
Dermatomyositis is a systemic connective tissue disorder characterized by
inflammatory and degenerative changes in the muscles, subcutaneous tissues
and skin. Symptoms may come and go, but the main symptom is muscle weakness,
most often in the hip and shoulder, usually accompanied by a rash. There is
often underlying cancer in 50% of adult cases. This disorder may possibly be
an autoimmune disease. (Autoimmune disorders are caused when the body's
natural defenses (antibodies, lymphocytes, etc.), against invading organisms
suddenly begin to attack healthy tissue.) (For more information on this
disorder, choose "Dermatomyositis" as your search term in the Rare Disease
Database).
Idiopathic Polymyositis is a systemic connective tissue disorder
characterized by inflammatory and degenerative changes in the muscles leading
to weakness and some degree of muscle atrophy. The areas primarily affected
are the hip, shoulder, and chest. (For more information on this disorder,
choose "Polymyositis" as your search term in the Rare Disease Database).
Distal Myopathy affects predominantly the small muscles of the
extremities. Onset is usually after age 40, with weakness and wasting of
small muscles of the hands. Autosomal dominant inheritance is thought to be
a cause.
Mixed Connective Tissue Disease (MCTD) is a rheumatic disease
characterized by overlapping features similar to those of systemic lupus
erythematous (SLE), scleroderma and polymyositis. The cause is unknown, but
it is suggested that the immune system may be involved. MCTD appears to be
more common than polymyositis and less common than scleroderma. Symptoms of
MCTD may include Raynaud's phenomena (cold and numbness of fingers),
arthritis, swollen hands, inflammatory proximal muscle weakness, dysfunction
of the esophagus, and lung disease. MCTD is often used to describe what may
be an overlapping group of connective tissue diseases that cannot be
diagnosed in more specific terms. (For more information on this disorder,
choose "MCTD" as your search term in the Rare Disease Database).
Oculopharyngeal Muscular Dystrophy usually occurs in adulthood.
Extraocular muscles are involved initially and the muscles used for
swallowing tend to become affected. The typical facial appearance,
especially drooping of the eyelids, resembles that found in myasthenia
gravis. The inheritance pattern often follows an autosomal dominant pattern.
However, occasional sporadic cases and cases with autosomal recessive
inheritance have occurred.
Therapies: Standard
Inclusion Body Myositis does not readily yield to treatment with steroids or
other immunosuppressive drugs. Researchers are trying to understand the
cause of IBM in order to develop more effective therapies. Other treatment
is symptomatic and supportive.
Therapies: Investigational
This disease entry is based upon medical information available through April
1989. 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 Inclusion Body Myositis, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
Inclusion Body Myositis Association
1420 Huron Ct.
Harrisburg, VA 22801
(703) 433-7935 (write or if calling, between the hours of 9:00-11:30 a.m.
and 6:30-7:30 p.m., please)
The National Arthritis and Musculoskeletal and Skin Diseases Information
Clearinghouse
Box AMS
Bethesda, MD 20892
(301) 495-4484
Muscular Dystrophy Association, National Office
3300 E. Sunrise Dr.
Tucson, AZ 85718
(602) 529-2000
References
MENDELIAN INHERITANCE IN MAN, 8th ed.: Victor A. McKusick; Johns Hopkins
University Press, 1986. Pp. 427.
ONOCLONAL ANTIBODY ANALYSIS OF MONOCUCLEAR CELLS IN MYOPATHIES. V:
IDENTIFICATION AND QUANTITATION OF T8+ CYTOTOXIC AND T8+ SUPPRESSOR CELLS.
K. Arahata, et. al; Ann. Neurol. (May, 1988, issue 23 (5)). Pp. 493-9.
INCLUSION BODY MYOSITIS: A CHRONIC PERSISTENT MUMPS MYOSITIS? S.M.
Chou; Hum. Pathol. (August, 1986, issue 17(8)). Pp. 765-77.
INCLUSION BODY MYOSITIS AND SYSTEMIC LUPUS ERYTHEMATOSUS. R.A. Yood, et.
al; J. Rheumatol. (June, 1985, issue 12(3)). Pp. 568-70.