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Copyright (C) 1986, 1987, 1988, 1990 National Organization for Rare
Disorders, Inc.

92:
Raynaud's Disease and Phenomenon

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.


Raynaud's Disease and Raynaud's Phenomenon are vascular disorders.  These
disorders are spasms of arterioles occurring especially in the fingers and
toes and occasionally in other acral parts of the body such as the nose and
tongue.  The intermittent attacks of pallor or cyanosis of the digits may be
precipitated by exposure to cold or by emotional upsets.  It may be either
idiopathic or secondary to other conditions.

Symptoms

Attacks of the disorders known as Raynaud's Disease and Phenomenon occur more
frequently in cold rather than hot weather.  Symptoms associated with
Raynaud's Disease (unless stated otherwise the use of the term Raynaud's
Disease will also include Raynaud's Phenomenon) may include a feeling of
numbness and coldness of the toes or fingers.  Only one or two fingertips may
be involved early in the course of the disease.  As the disorder progresses,
all the fingers down to the distal palm may be affected, although the thumbs
are rarely involved.  Patients may experience sensory changes accompanying
the vasomotor manifestations such as an aching pain, tingling feeling, or
throbbing in the afflicted digits.  There may be the sensation of tightness
or pins and needles.

Onset is usually in the first or second decade of life.  The vasospasm
attacks may last for minutes to hours, but are rarely severe enough to result
in gross tissue loss.  The intermittent attacks of cyanosis or blanching may
be precipitated by exposure to cold or by emotional upsets.  The color
changes in Raynaud's Phenomenon may be either triphasic (pallor, cyanosis,
and redness followed by reactive hyperemia) or biphasic (cyanosis followed by
reactive hyperemia).  Rewarming the affected digits results in a return to
normal color and sensation.

Raynaud's Disease is much rarer than Raynaud's Phenomenon.  Raynaud's
Disease is usually benign, causing only mild discomfort and progressing very
slightly over the years.  In some cases, however, there is a rapid
progression of the disorder which may result in sclerodactyly (loss of
subcutaneous tissue) or small painful ulcers may appear on the tips of the
affected digits.

Raynaud's Disease is suspected when there appears to be no evidence for
an underlying cause, while Raynaud's Phenomenon occurs as a secondary
characteristic to another disorder.

Causes

The cause of Raynaud's Disease is generally unknown.  It can occur as a
symptom of other disorders or it may possibly be a hereditary disorder.  It
may also be related to a local sensitivity of the digital vessels to cold, or
the bilateral paroxysmal contraction of arteries and arterioles of the
fingers or toes may occur without apparent cause.  The attacks of the disease
may be precipitated by exposure to cold or occasionally by emotional upsets.

Raynaud's Phenomenon may also occur as a secondary characteristic to
other disorders.  It may be related to conditions such as connective tissue
disorders (e.g., scleroderma or systemic lupus erythematosus), neurogenic
lesions (including the thoracic outlet syndromes), drug intoxications (ergot
and methysergide), dysproteinemias, myxedema, primary pulmonary hypertension,
and trauma.  (For related information, choose "scleroderma" and "lupus" as
your search terms in the Rare Disease Database.)

Research is currently ongoing at the University of Carolina to identify
criteria that will be useful to predict whether or not Raynaud's Phenomenon
will develop into Scleroderma.

Affected Population

Raynaud's Disease and Raynaud's Phenomenon occur predominantly in women.  It
is less common before puberty and after the age of forty years.

Therapies:  Standard

Therapy for secondary forms of this disorder is dependant upon recognition
and treatment of the underlying disturbance.  Mild cases of Raynaud's Disease
may be controlled by protecting the patient from exposure to cold and by the
use of mild sedatives such as phenobarbital.  Since nicotine is a
vasoconstrictor, the patient must stop smoking.  Phenoxybenzamine,
guanethidine, reserpine, and methyldopa have also been useful in treatment.
Occasionally, regional sympathectomy (surgical removal of the sheath of an
artery containing the sympathetic nerve fibers which leads to improved
vasodilatation) may benefit patients.  Sympathectomy generally produces
better results in patients with Raynaud's disease than in those with
Raynaud's Phenomenon.

Therapies:  Investigational

The disordered blood flow in the fingers of Raynaud's disease may be
treatable with the drug ketanserin, an antagonist of serotonin.  More
research is needed before this drug will be available for more general use.

Recent research by the U.S. Army indicates that the body may be tricked
into circulating blood to the hands even in cold weather.  Three to six times
each day Raynaud's patients sat indoors with their hands submerged in warm
water.  Then they were put into a cold environment where their body was
exposed to cold temperatures except their hands, which were submerged in an
ice chest filled with warm water.  After 50 rounds of this treatment, 150
test subjects were able to go out in cold weather without losing circulation
in their hands.  More research is needed to determine if this treatment will
work for many people over an extended period of time.

Scientists are studying a new orphan drug, Iloprost, for treatment of
Raynaud's Phenomenon when it occurs along with Scleroderma.  The drug is
manufactured by Berflex Laboratories.  More research is needed to determine
the safety and effectiveness of this experimental treatment.

New treatment agents are being tested for Raynaud's Disease.  They
include the calcium antagonist, nifedipine and the ACE-inhibitor, captopril
(both manufactured by Bristol-Myers Squibb; the alpha-blocker, Thymoxamine
(Parke-Davis' Opilon); and Janssen's serotonin receptor antagonist,
ketanserin, according to a recent editorial in the British Medical Journal
(March 3, 1990, p. 553).

This disease entry is based upon medical information available through
April 1990.  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 Raynaud's Disease and Phenomenon, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     [phone]

     The Raynaud's Association Trust
     112 Crewe Rd.
     Alsager, Cheshire, ST7 2JA
     England

     NIH/National Heart, Lung, and Blood Institute
     9000 Rockville Pike
     Bethesda, MD  20892
     [phone]

     American Heart Association
     7320 Greenville Ave.
     Dallas, TX  75231
     [phone]

References

CECIL TEXTBOOK OF MEDICINE, 18th ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Eds.:  W. B. Saunders Co., 1988.  Pp. 369, 375-7.

THE MERCK MANUAL 15th ed:  R. Berkow, et al: eds; Merck, Sharp & Dohme
Research Laboratories, 1987.  P. 562.