$Unique_ID{BRK03491}
$Pretitle{}
$Title{Arteritis, Takayasu}
$Subject{Arteritis, Takayasu Pulseless Disease Takayasu Disease Young Female
Disease Reverse Coarction Martorell Syndrome Brachiocephalic Ischemia Aortic
Arch Syndrome Idiopathic Arteritis of Takayasu}
$Volume{}
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Copyright (C) 1986, 1989 National Organization for Rare Disorders, Inc.

86:
Arteritis, Takayasu

** IMPORTANT **
It is possible that the main title of the article (Takayasu Arteritis) is
not the name you expected.  Please check the SYNONYMS listing to find
alternate names and disorder subdivisions covered by the article.

Synonyms

     Pulseless Disease
     Takayasu Disease
     Young Female Disease
     Reverse Coarction
     Martorell Syndrome
     Brachiocephalic Ischemia
     Aortic Arch Syndrome
     Idiopathic Arteritis of Takayasu

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.

Arteritis is an inflammation of the blood vessels.  Takayasu Arteritis,
also known as Pulseless Disease, is a progressive polyarteritis of unknown
cause which affects the largest central blood vessels such as the aorta
and its branches.  A collagen vascular disease, Takayasu Arteritis affects
mainly women.  The disorder becomes clinically evident from a narrowing of
the vessels which may result in the reduction of blood flow to the head and
arms with loss of the major pulses.

Symptoms

Takayasu's Arteritis is characterized by easy fatigability of the arms,
headache, vertigo and dizziness.  Aphasia which is impairment of the
patient's ability to communicate and/or comprehend may also be present.
Possible ocular manifestations include episodes of blindness, amblyopia
during which time the patient's vision is dimmed, or an abnormal intolerance
to light known as photophobia.  The muscles involved in chewing may become
flaccid.  Weakness or a temporary loss of consciousness upon exercising after
a period of standing (syncopal attacks) may occur.

Clinical manifestations may include atrophy of the muscles and soft
tissues of the face, degeneration of the cartilage which makes up the nasal
septum, and cataracts.  The pulse in the upper extremities, carotid and
superficial temporal arteries may be lost.  Blood pressure in the arms may be
imperceptible and there may be hypertension in the lower extremities.
Systolic murmurs, erythema nodosum, or fever may be present.  Raynaud's
Phenomenon, which is a spasm of arterioles, especially in the fingers and
toes, characterized by intermittent pallor or cyanosis of the skin
precipitated by exposure to cold or by emotional upset is also common.

Most patients develop cardiac and cerebral insufficiencies as a result of
Takayasu's Arteritis.  The prognosis is more favorable with a slower
progression of the disease and the development of adequate collateral or
secondary circulation.  A ruptured aneurysm may sometimes occur.

Causes

The cause of Takayasu's Arteritis is not clear at this time.  It may be
related to an autoimmunological mechanism or possibly a hypersensitivity
reaction.

Affected Population

Although the disorder known as Takayasu's Arteritis was first described in
the Orient, it is found worldwide.  The disease predominantly affects women
between the ages of 10 and 50 years.

Related Disorders

Other vasculitic disorders include Henoch-Schonlein syndrome, Pseudomonas
septicemia, erythema nodosum, polyarteritis nodosa, and giant-cell arteritis.

Therapies:  Standard

In the early stages, treatment with corticosteroids may arrest the disease.
Heparin during ischemic episodes and reconstructive surgery for shunting of
blood around occluded blood vessels may be helpful.

Therapies:  Investigational

This disease entry is based upon medical information available through March
1987.  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 Takayasu Arteritis, please contact:

     National Organization for Rare Disorders
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     (203) 746-6518

     NIH/National Heart, Lung and Blood Institute
     9000 Rockville Pike
     Bethesda, MD  20892
     (301) 496-4236

References

THE MERCK MANUAL 15th ed.:  R. Berkow, et al:  eds; Merck, Sharp & Dohme
Research Laboratories, 1987.  P. 546.

CECIL TEXTBOOK OF MEDICINE, 18th ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Eds.:  W. B. Saunders Co., 1988.  P. 2164.