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- $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{}
- $Log{}
-
- 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.
-
-