Raynaud's phenomenon

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Raynaud's phenomenon

Classification and external resources

Hands with Raynaud's phenomenon

ICD-10 I73.0

ICD-9 443.0

DiseasesDB 25933

eMedicine med/1993

MeSH D011928

In medicine, Raynaud's phenomenon (pronounced /reɪˈnoʊz/, US dict: rā·nōz′) is a

vasospastic disorder causing discoloration of the fingers, toes, and occasionally other areas.
This condition can also cause nails to become brittle with longitudinal ridges. Named for
French physician Maurice Raynaud (1834–1881), the phenomenon is believed to be the result
of vasospasms that decrease blood supply to the respective regions. Emotional stress and cold
are classic triggers of the phenomenon.
It comprises both Raynaud's disease (also known as "Primary Raynaud's phenomenon")
where the phenomenon is idiopathic,[1] and Raynaud's syndrome (secondary Raynaud's),
where it is caused by some other instigating factor. Measurement of hand-temperature
gradients is one tool used to distinguish between the primary and secondary forms.[2]
It is possible for the primary form to progress to the secondary form.[3]
In extreme cases, the secondary form can progress to necrosis or gangrene of the fingertips.
Raynaud's phenomenon is an exaggeration of vasomotor responses to cold or emotional
stress. More specifically, it is a hyperactivation of the sympathetic system causing extreme
vasoconstriction of the peripheral blood vessels, leading to tissue hypoxia. Chronic, recurrent
cases of Raynaud phenomenon can result in atrophy of the skin, subcutaneous tissues, and
muscle. In rare cases it can cause ulceration and ischemic gangrene.[4]

Contents
[hide]
• 1 Symptoms
• 2 Prevalence
• 3 Epidemiology
○ 3.1 Primary Raynaud's (disease)
○ 3.2 Secondary Raynaud's (syndrome)
• 4 Examination
• 5 Treatment
○ 5.1 General care
○ 5.2 Emergency measures
○ 5.3 Drug therapy
○ 5.4 Surgical Intervention
○ 5.5 Alternative and Experimental (Research) Approaches
• 6 See also
• 7 Footnotes
• 8 External links

[edit] Symptoms
The condition can cause pain within the affected extremities, discoloration (paleness) and
sensations of cold and/or numbness. This can often be distressing to those who are not
diagnosed, and sometimes it can be obstructive. If someone with Raynaud's is placed in too
cold a climate, it could potentially become dangerous.
The symptoms include several cyclic color changes:
1. When exposed to cold temperatures, the blood supply to the fingers or toes, and in
some cases the nose or earlobes, is markedly reduced; the skin turns pale or white
(called pallor), and becomes cold and numb.
2. When the oxygen supply is depleted, the skin colour turns blue (called cyanosis).
 3. These events are episodic, and when the episode subsides or the area is warmed, the
blood flow returns and the skin colour first turns red (rubor), and then back to normal,
often accompanied by swelling and tingling.
All three colour changes are observed in classic Raynaud's. However, not all patients see all
of the aforementioned colour changes in all episodes, especially in milder cases of the
condition. Symptoms are thought to be due to reactive hyperemias of the areas deprived of
blood flow.
In pregnancy, this sign normally disappears due to increased surface blood flow. Raynaud's
has also occurred in breastfeeding mothers, causing nipples to turn white and become
extremely painful.[5] Nifedipine, a calcium channel blocker and vasodilator was recommended
to increase blood flow to the extremities and noticeably relieved pain to the breast, in an
extremely small study group.[6]
[edit] Prevalence
The phenomenon is more common in women than men, with the Framingham Study finding
that 5% of men and 8% of women suffer from it.[verification needed]
[edit] Epidemiology
It is important to distinguish Raynaud's disease from syndrome. In order to diagnose these
two forms of Raynaud's, a doctor may look for signs of arthritis or vasculitis, and may
conduct a number of laboratory tests.
[edit] Primary Raynaud's (disease)
Raynaud's disease, or "Primary Raynaud's", is diagnosed if the symptoms are idiopathic,
that is, they occur by themselves and not in association with other diseases. Some refer to
Primary Raynaud's disease as "being allergic to coldness". It often develops in young women
in their teens and early adulthood. Primary Raynaud's is thought to be at least partly
hereditary, although specific genes have not yet been identified.[7]
Smoking worsens frequency and intensity of attacks, and there is a hormonal component.
Caffeine also worsens the attacks. Sufferers are more likely to have migraine and angina than
controls.
[edit] Secondary Raynaud's (syndrome)
Raynaud's syndrome, or "Secondary Raynaud's", occurs secondary to a wide variety of
other conditions. Secondary Raynaud's has a number of associations:
• Connective tissue disorders:
○ scleroderma[8]
○ systemic lupus erythematosus
○ rheumatoid arthritis
○ Sjögren's syndrome
○ dermatomyositis
○ polymyositis
○ mixed connective tissue disease
○ cold agglutinin disease
○ Ehlers-Danlos Syndrome
• Eating disorders
 ○ anorexia nervosa
• Obstructive disorders
○ atherosclerosis
○ Buerger's disease
○ Takayasu's arteritis
○ subclavian aneurysms
○ thoracic outlet syndrome
• Drugs
○ Beta-blockers
○ cytotoxic drugs - particularly chemotherapeutics and most especially
bleomycin
○ ciclosporin
○ ergotamine
○ sulfasalazine
○ anthrax vaccines whose primary ingredient is the Anthrax Protective Antigen
• Occupation
○ jobs involving vibration, particularly drilling, suffer from vibration white
finger
○ exposure to vinyl chloride, mercury
○ exposure to the cold (e.g. by working packing frozen food)
• Others
○ hypothyroidism
○ cryoglobulinemia
○ malignancy
○ reflex sympathetic dystrophy
○ carpal tunnel syndrome
○ Magnesium Deficiency
○ Erythromelalgia, (the opposite of Raynaud's, with hot and warm extremities)
often co-exists in patients with Raynaud's)[9]
It is important to realize that Raynaud's can herald these diseases by periods of more than 20
years in some cases, making it effectively their first presenting symptom. This can be the case
in the CREST syndrome, of which Raynaud's is a part.
Patients with Secondary Raynaud's can also have symptoms related to their underlying
diseases. Raynaud's phenomenon is the initial symptom that presents for 70% of patients with
scleroderma, a skin and joint disease.
When Raynaud's phenomenon is limited to one hand or one foot, it is referred to as Unilateral
Raynaud's. This is an uncommon form, and it is always secondary to local or regional
vascular disease. It commonly progresses within several years to affect other limbs as the
vascular disease progresses.[10]
[edit] Examination
A careful medical history will often reveal whether the condition is primary or secondary.
Once this has been established, an examination is largely to identify or exclude possible
secondary causes.
• Digital artery pressure: pressures are measured in the arteries of the fingers before and
after the hands have been cooled. A decrease of at least 15 mmHg is diagnostic
(positive).
• Doppler ultrasound: to assess blood flow.
• Full blood count: this can reveal a normocytic anaemia suggesting the anaemia of
chronic disease or renal failure.
• Blood test for urea and electrolytes: this can reveal renal impairment.
• Thyroid function tests: this can reveal hypothyroidism.
• An autoantibody screen, tests for rheumatoid factor, Erythrocyte sedimentation rate
and C-reactive protein, which may reveal specific causative illnesses or a generalised
inflammatory process.
• Nail fold vasculature: this can be examined under the microscope.
[edit] Treatment
Treatment options are dependent on the type of Raynaud's present. Raynaud's syndrome is
treated primarily by addressing the underlying cause, but includes all options for Raynaud's
disease as well. Treatment of primary Raynaud's focuses on avoiding triggers.
[edit] General care
• Avoid environmental triggers, e.g. cold, vibration, etc. Emotional stress is another
recognized trigger; although the various sources of stress can not all be avoided, it is
possible to learn healthier, more effective ways of dealing with them, which will
reduce stress and its damaging physical effects overall.
• Keep your hands, feet and head warm—especially your fingers, toes, ears and nose—
by wearing mittens, insulated footwear, a ski mask; by using hand and foot warmers,
etc.
• Quit smoking.
• Avoid caffeine and other stimulants and vasoconstrictors that have not been
prescribed to you by your doctor. Read product labels; caffeine is found not only in
coffee and tea, stay-awake pills, many soft drinks and candies, but also in some
cosmetics, soaps and shampoos(shower shock caffinated bar and alpecin sampoo).
• Make sure all your doctors know about all the medicines you take and about all the
OTC remedies you use, especially hormones and drugs that regulate hormones, such
as hormonal contraception, so that these professionals can make an assessment of
your chemical regimen and make any changes that may be indicated. Contraception
which is low in estrogen is preferable, and the progesterone only pill is often
prescribed for women with Raynaud's.
• If you are diabetic, follow your diabetes treatment plan.
[edit] Emergency measures
• If white finger (Raynaud's) occurs unexpectedly and a source of warm water is
available, allow tepid to slightly warm water to run over the affected digits while you
 gently massage the area. Continue this process until the white area returns to its
normal, healthy color.
• If triggered by exposure in a cold environment, and no warm water is available, place
the affected digits in a warm body cavity - arm pit, crotch, or even in the mouth. Keep
the affected area warm at least until the whiteness returns to its normal, healthy color.
Get out of the cold as soon as possible.
[edit] Drug therapy
• Treatment for Raynaud's phenomenon may include prescription medicines that dilate
blood vessels, such as calcium channel blockers (nifedipine) or diltiazem.[11][12] It has
the usual common side effects of headache, flushing, and ankle edema; but these are
not typically of sufficient severity to require cessation of treatment.[13]
• There is some evidence that Angiotensin II receptor antagonists (often Losartan)
reduce frequency and severity of attacks,[14] and possibly better than nifedipine.[15]
• Alpha-1 adrenergic blockers such as prazosin can be used to control Raynaud's
vasospasms under supervision of a health care provider.[16]
• In a study published in the November 8, 2005 issue of Circulation, sildenafil (Viagra)
improved both microcirculation and symptoms in patients with secondary Raynaud's
phenomenon resistant to vasodilatory therapy. The authors, led by Dr Roland Fries
(Gotthard-Schettler-Klinik, Bad Schönborn, Germany), report: "In the present study,
capillary blood flow was severely impaired and sometimes hardly detectable in
patients with Raynaud's phenomenon. Sildenafil led to a more than 400% increase of
flow velocity."[17]
• Fluoxetine, a selective serotonin reuptake inhibitor, and other antidepressant
medications may reduce the frequency and severity of episodes if caused mainly by
psychological stress.
[edit] Surgical Intervention
• In severe cases, a sympathectomy[18] procedure can be performed. Here, the nerves
that signal the blood vessels of the fingertips to constrict are surgically cut.
Microvascular surgery of the affected areas is another possible therapy. Infusions of
prostaglandins, e.g. prostacyclin, may be tried, with amputation in exceptionally
severe cases.
• A more recent treatment for severe Raynaud's is the use of Botox. The 2009 article[19]
studied 19 patients ranging in age from 15 to 72 years with severe Raynaud's
phenomenon of which 16 patients (84%) reported pain reduction at rest. 13 patients
reported immediate pain relief, 3 more had gradual pain reduction over 1-2 months.
All 13 patients with chronic finger ulcers healed within 60 days. Only 21% of the
patients required repeated injections. A 2007 article[20] describes similar improvement
in a series of 11 patients. All patients had significant relief of pain.
[edit] Alternative and Experimental (Research) Approaches
• The extract of the Ginkgo biloba leaves (Egb 761, 80 mg) may reduce frequency of
attacks.[21]
• Two separate gels combined on the fingertip (somewhat like two-part epoxy, they
cannot be combined before use because they will react) increased blood flow in the
fingertips by about three times. One gel contained 5% sodium nitrite and the other
contained 5% ascorbic acid. The milliliter of combined gel covered an area of ~3 cm².
The gel was wiped off after a few seconds.[22]
 • Piracetam, a nootropic drug, can be useful as a long-term treatment for vasospastic
disorders.
• Arginine, which increase nitrous oxide acts as a vasodilator
• Milder cases of Raynaud's can often be addressed by biofeedback[23] or other
techniques to help control involuntary body functions like skin temperature.
• Fish oil supplements which contain long-chain omega-3 fatty acids may help to
control symptoms of primary Raynaud's. There are few studies in the medical
literature dealing with this subject. However, in one 1989 controlled, double-blinded
study of 32 patients,[24] consumption of roughly 6.5 grams of long chain omega-3 fatty
acids in the form of fish oil significantly increased the time to onset or entirely
prevented symptoms in response to cold in patients with primary Raynaud's. Lower
doses of fish oil such as may be commonly available from commercial vendors have
not been studied and may not be as effective.
[edit] See also
• CREST syndrome
• Circulatory system
• Vasospasm
• Cryopedis
[edit] Footnotes
1. ^ Raynaud disease at Dorland's Medical Dictionary
2. ^ Anderson ME, Moore TL, Lunt M, Herrick AL (March 2007). "The 'distal-dorsal
difference': a thermographic parameter by which to differentiate between primary and
secondary Raynaud's phenomenon". Rheumatology 46 (3): 533–8.
doi:10.1093/rheumatology/kel330. PMID 17018538.
3. ^ Hirschl M, Hirschl K, Lenz M, Katzenschlager R, Hutter HP, Kundi M (June 2006).
"Transition from primary Raynaud's phenomenon to secondary Raynaud's phenomenon
identified by diagnosis of an associated disease: results of ten years of prospective
surveillance". Arthritis and Rheumatism 54 (6): 1974–81. doi:10.1002/art.21912.
PMID 16732585.
4. ^ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.;
Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease. St. Louis, Mo:
Elsevier Saunders. p. 542. ISBN 0-7216-0187-1.
5. ^ Holmen OL, Backe B (2009). "An underdiagnosed cause of nipple pain presented on a
camera phone". BMJ 339: b2553. doi:10.1136/bmj.b2553.
6. ^ Anderson JE, Held N, Wright K (April 2004). "Raynaud's phenomenon of the nipple: a
treatable cause of painful breastfeeding". Pediatrics 113 (4): e360–4.
doi:10.1542/peds.113.4.e360. PMID 15060268.
7. ^ Pistorius MA, Planchon B, Schott JJ, Lemarec H (February 2006). "[Heredity and genetic
aspects of Raynaud's disease"] (in French). Journal Des Maladies Vasculaires 31 (1): 10–5.
PMID 16609626. http://www.masson.fr/masson/MDOI-JMV-01-2006-31-1-0398-0499-
101019-200517601.
8. ^ Gayraud M (January 2007). "Raynaud's phenomenon". Joint, Bone, Spine 74 (1): e1–8.
doi:10.1016/j.jbspin.2006.07.002. PMID 17218139.
9. ^ Berlin AL, Pehr K (March 2004). "Coexistence of erythromelalgia and Raynaud's
phenomenon". Journal of the American Academy of Dermatology 50 (3): 456–60.
doi:10.1016/S0190-9622(03)02121-2. PMID 14988692.
 10.^ Priollet P (October 1998). "[Raynaud's phenomena: diagnostic and treatment study]" (in
French). La Revue Du Praticien 48 (15): 1659–64. PMID 9814067.
11.^ Kahan A, Weber S, Amor B, Saporta L, Hodara M, Degeorges M (April 1981). "Nifedipine
and Raynaud's phenomenon". Annals of Internal Medicine 94 (4 pt 1): 546. PMID 7212523.
12.^ Kahan A, Weber S, Amor B, Saporta L, Hodara M, Degeorges M (April 1982).
"[Controlled study of nifedipine in the treatment of Raynaud's phenomenon]" (in French).
Revue Du Rhumatisme et Des Maladies Ostéo-articulaires 49 (5): 337–43. PMID 6285445.
13.^ Smith CR, Rodeheffer RJ (January 1985). "Raynaud's phenomenon: pathophysiologic
features and treatment with calcium-channel blockers". The American Journal of Cardiology
55 (3): 154B–157B. doi:10.1016/0002-9149(85)90625-3. PMID 3881908.
14.^ Pancera P, Sansone S, Secchi S, Covi G, Lechi A (November 1997). "The effects of
thromboxane A2 inhibition (picotamide) and angiotensin II receptor blockade (losartan) in
primary Raynaud's phenomenon". Journal of Internal Medicine 242 (5): 373–6.
doi:10.1046/j.1365-2796.1997.00219.x. PMID 9408065.
15.^ Dziadzio M, Denton CP, Smith R, et al. (December 1999). "Losartan therapy for Raynaud's
phenomenon and scleroderma: clinical and biochemical findings in a fifteen-week,
randomized, parallel-group, controlled trial". Arthritis and Rheumatism 42 (12): 2646–55.
doi:10.1002/1529-0131(199912)42:12<2646::AID-ANR21>3.0.CO;2-T. PMID 10616013.
16.^ Waldo R (March 1979). "Prazosin relieves Raynaud's vasospasm". JAMA 241 (10): 1037.
doi:10.1001/jama.241.10.1037. PMID 762741.
17.^ Fries R, Shariat K, von Wilmowsky H, Böhm M (November 2005). "Sildenafil in the
treatment of Raynaud's phenomenon resistant to vasodilatory therapy". Circulation 112 (19):
2980–5. doi:10.1161/CIRCULATIONAHA.104.523324 (inactive 2010-02-15).
PMID 16275885. http://circ.ahajournals.org/cgi/content/abstract/112/19/2980.
18.^ Wang WH, Lai CS, Chang KP, et al. (October 2006). "Peripheral sympathectomy for
Raynaud's phenomenon: a salvage procedure". The Kaohsiung Journal of Medical Sciences
22 (10): 491–9. doi:10.1016/S1607-551X(09)70343-2. PMID 17098681.
19.^ Neumeister MW, Chambers CB, Herron MS, et al. (July 2009). "Botox therapy for
ischemic digits". Plastic and Reconstructive Surgery 124 (1): 191–201.
doi:10.1097/PRS.0b013e3181a80576. PMID 19568080.
20.^ Van Beek AL, Lim PK, Gear AJ, Pritzker MR (January 2007). "Management of vasospastic
disorders with botulinum toxin A". Plastic and Reconstructive Surgery 119 (1): 217–26.
doi:10.1097/01.prs.0000244860.00674.57. PMID 17255677.
21.^ Muir AH, Robb R, McLaren M, Daly F, Belch JJ (2002). "The use of Ginkgo biloba in
Raynaud's disease: a double-blind placebo-controlled trial". Vascular Medicine 7 (4): 265–7.
doi:10.1191/1358863x02vm455oa. PMID 12710841.
22.^ Tucker AT, Pearson RM, Cooke ED, Benjamin N (November 1999). "Effect of nitric-
oxide-generating system on microcirculatory blood flow in skin of patients with severe
Raynaud's syndrome: a randomised trial". Lancet 354 (9191): 1670–5. doi:10.1016/S0140-
6736(99)04095-7. PMID 10568568.
23.^ Karavidas MK, Tsai PS, Yucha C, McGrady A, Lehrer PM (September 2006). "Thermal
biofeedback for primary Raynaud's phenomenon: a review of the literature". Applied
Psychophysiology and Biofeedback 31 (3): 203–16. doi:10.1007/s10484-006-9018-2.
PMID 17016765.
24.^ DiGiacomo RA, Kremer JM, Shah DM (February 1989). "Fish-oil dietary supplementation
in patients with Raynaud's phenomenon: a double-blind, controlled, prospective study". The
American Journal of Medicine 86 (2): 158–64. doi:10.1016/0002-9343(89)90261-1.
PMID 2536517.

[edit] External links

 • Overview at National Institutes of Health
• Overview at Medical College of Wisconsin
• Raynaud's Disease at healthatoz.com
• Raynaud's Phenomenon at YouTube
• Raynaud's Disease at YouTube
• Video at YouTube
• What Is Raynaud's Disease at National Heart, Lung, and Blood Institute
• Raynaud's Disease at Mayo Clinic
• Raynaud's & Scleroderma Association, a national charity and self help organisation,
committed to supporting patients and carers who have these conditions
• Bakst R, Merola JF, Franks AG, Sanchez M (October 2008). "Raynaud's
phenomenon: pathogenesis and management". Journal of the American Academy of
Dermatology 59 (4): 633–53. doi:10.1016/j.jaad.2008.06.004. PMID 18656283.
Retrieved from "http://en.wikipedia.org/wiki/Raynaud%27s_phenomenon"
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Rheumatology | Syndromes | Vascular-related cutaneous conditions
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Raynaud's Phenomenon & Raynaud's

Syndrome - your questions answered

What Is Raynaud's Phenomenon?

Raynaud's is a disorder that affects the blood vessels in the fingers, toes, ears, and nose. This
disorder is characterised by episodic attacks, called vasospastic attacks, that cause the blood
vessels in the fingers and toes to constrict. Raynaud's can occur on its own, or it can be
secondary to another condition such as scleroderma or lupus.
Is it Raynaud's syndrome, Raynaud's phenomenon or
Raynaud's disease?
There is a lot of confusion on the internet regarding Raynaud's so we thought it was
important to spell out the differences in terminology early on in this article which we will
expand on later. The name Raynaud's Disease was given to this condition by a French doctor,
Maurice Raynaud in 1862 when he first described the phenomenon. The name Raynaud's
disease has more recently been replaced with Raynaud's on its own or Raynaud's
phenomenon. Raynaud's can be subdivided into primary and secondary conditions and the
secondary form is also known as Raynaud's syndrome.
Who does Raynauds affect?
Although estimates vary, recent surveys show that Raynaud's syndrome may affect 5 to 10
percent of the general population. Women are more likely than men to have the disorder.
Raynaud's syndrome appears to be more common in people who live in colder climates.
However, people with the disorder who live in milder climates may have more attacks during
periods of colder weather.
What Happens During an Attack?
For most people, an attack is usually triggered by exposure to cold or emotional stress. In
general, attacks affect the fingers or toes but may affect the nose, lips, or ear lobes.
Reduced Blood Supply to the Extremities
When a person is exposed to cold, the body's normal response is to slow the loss of heat and
preserve its core temperature. To maintain this temperature, the blood vessels that control
blood flow to the skin surface move blood from arteries near the surface to veins deeper in
the body. For people who have Raynaud's syndrome, this normal body response is intensified
by the sudden spasmodic contractions of the small blood vessels (arterioles) that supply blood
to the fingers and toes. The arteries of the fingers and toes may also collapse. As a result, the
blood supply to the extremities is greatly decreased, causing a reaction that includes skin
discoloration and other changes.
Changes in Skin Colour and Sensation
Once the attack begins, a person may experience three phases of skin colour changes (white,
blue, and red) in the fingers or toes. The order of the changes of colour is not the same for all
people, and not everyone has all three colours. Pallor (whiteness) may occur in response to
spasm of the arterioles and the resulting collapse of the digital arteries. Cyanosis (blueness)
may appear because the fingers or toes are not getting enough oxygen-rich blood. The fingers
or toes may also feel cold and numb. Finally, as the arterioles dilate (relax) and blood returns
to the digits, rubor (redness) may occur. As the attack ends, throbbing and tingling may occur
in the fingers and toes. An attack can last from less than a minute to several hours.
How Is Raynaud's Classified?
Doctors classify Raynaud's as either the primary or the secondary form (also known as
Raynaud's Syndrome). In medical terms, "primary Raynaud’s" may also be called Raynaud's
disease or idiopathic Raynaud's. The terms idiopathic and primary both mean that the cause is
unknown.
Primary Raynaud's or Raynaud's disease
Most people who have Raynaud's have the primary form (the milder version). A person who
has primary Raynaud's has no underlying disease or associated medical problems. More
women than men are affected, and approximately 75 percent of all cases are diagnosed in
women who are between 15 and 40 years old.
People who have only vasospastic attacks for several years, without involvement of other
body systems or organs, rarely have or will develop a secondary disease (that is, a connective
tissue disorder such as scleroderma) later. Several researchers who studied people who
appeared to have primary Raynaud's over long periods of time found that less than 9 percent
of these people developed a secondary disease.
Secondary Raynaud's or Raynaud's Syndrome
Although secondary Raynaud's (Raynaud's syndrome) is less common than the primary form,
it is often a more complex and serious disorder. Secondary means that patients have an
underlying disease or condition that causes Raynaud's Syndrome. Connective tissue diseases
are the most common cause of Raynaud's syndrome. Some of these diseases reduce blood
flow to the digits by causing blood vessel walls to thicken and the vessels to constrict too
easily. Raynaud's syndrome is seen in approximately 85 to 95 percent of patients with
scleroderma and mixed connective tissue disease, and it is present in about one-third of
patients with systemic lupus erythematosus. Raynaud's syndrome also can occur in patients
who have other connective tissue diseases, including Sjogren's syndrome, dermatomyositis
and polymyositis.
Possible causes of Raynaud's syndrome, other than connective tissue diseases, are carpal
tunnel syndrome and obstructive arterial disease (blood vessel disease). Some drugs,
including beta-blockers (used to treat high blood pressure), ergotamine preparations (used for
migraine headaches), certain agents used in cancer chemotherapy, and drugs that cause
vasoconstriction (such as some over-the-counter cold medications and narcotics), are linked
to Raynaud's syndrome.
People in certain occupations may be more vulnerable to secondary Raynaud's syndrome.
Some workers in the plastics industry (who are exposed to vinyl chloride) develop a
scleroderma-like illness, of which Raynaud's syndrome can be a part. Workers who operate
vibrating tools can develop a type of Raynaud's syndrome called vibration-induced white
finger (VWF) or hand arm vibration syndrome (HAVS).
When Raynaud's syndrome is associated with scleroderma (Systemic Sclerosis) problems
may involve skin ulcers (sores) or gangrene (tissue death) in the fingers or toes. Painful ulcers
and gangrene are fairly common and can be difficult to treat. In addition, a person may
experience heartburn or difficulty in swallowing. These two problems are caused by
weakness in the muscle of the oesophagus (the tube that takes food and liquids from the
mouth to the stomach) that can occur in people with connective tissue diseases. However, the
more serious consequences are caused by involvement of the lungs,heart, kidneys and blood
vessels.
How Does a Doctor Diagnose Raynaud's ?
If a doctor suspects Raynaud's, he or she will ask the patient for a detailed medical history.
The doctor will then examine the patient to rule out other medical problems. The patient
might have a vasospastic attack during the visit, which makes it easier for the doctor to
diagnose Raynaud's. Most doctors find it fairly easy to diagnose Raynaud's phenomenon but
more difficult to identify the form of the disorder.
Nailfold capillaroscopy (study of capillaries under a microscope) can help the doctor
distinguish between primary and secondary Raynaud's (Raynaud's syndrome). During this
test, the doctor puts a drop of oil on the patient's nailfolds, the skin at the base of the
fingernail. The doctor then examines the nailfolds under a microscope to look for
abnormalities of the tiny blood vessels called capillaries. If the capillaries are enlarged or
deformed, the patient may have a connective tissue disease and should be referred to a
specialist centre.
The doctor may also order two particular blood tests, an antinuclear antibody test (ANA) and
an erythrocyte sedimentation rate (ESR). The ANA test determines whether the body is
producing special proteins (antibodies) often found in people who have connective tissue
diseases or other autoimmune disorders. The ESR test is a measure of inflammation in the
body and tests how fast red blood cells settle out of unclotted blood. Inflammation in the
body causes an elevated ESR.
Diagnostic Criteria for Raynaud's
Primary Raynaud's Phenomenon
• Periodic vasospastic attacks of pallor or cyanosis (some doctors include the additional
criterion of the presence of these attacks for at least 2 years)
• Normal nailfold capillary pattern
• Negative antinuclear antibody test
• Normal erythrocyte sedimentation rate
• Absence of pitting scars or ulcers of the skin, or gangrene (tissue death) in the fingers
or toes
Secondary Raynaud's Syndrome
• Periodic vasospastic attacks of pallor and cyanosis
• Abnormal nailfold capillary pattern
• Positive antinuclear antibody test
 • Abnormal erythrocyte sedimentation rate
• Presence of pitting scars or ulcers of the skin, or gangrene in the fingers or toes
What Is the Treatment for Raynaud's?
The aims of treatment are to reduce the number and severity of attacks and to prevent tissue
damage and loss in the fingers and toes. Most doctors are conservative in treating patients
with Raynaud's that is, they recommend non-drug treatments and self-help measures first.
Doctors may prescribe medications for some patients, usually those with secondary
Raynaud's syndrome. In addition, patients are treated for any underlying disease or condition
that causes secondary Raynaud's syndrome.
Non-drug Treatments and Self-Help Measures
Several nondrug treatments and self-help measures can decrease the severity of Raynaud's
attacks and promote overall well-being.
Take action during an attack
An attack should not be ignored. Its length and severity can be lessened by a few simple
actions. The first and most important action is to warm the hands or feet. In cold weather,
people should go indoors. Running warm water over the fingers or toes or soaking them in
bowls of alternate cool and warm water will warm them. Taking time to relax will further
help to end the attack. If a stressful situation triggers the attack, a person can help stop the
attack by getting out of the stressful situation and relaxing. People who are trained in
biofeedback can use this technique along with warming the hands or feet in water to help
lessen the attack.
Keep warm
It is important not only to keep the extremities warm but also to avoid chilling any part of the
body. In cold weather, people with Raynaud's must pay particular attention to dressing.
Several layers of loose clothing, socks, hats, and gloves or mittens are recommended. A hat is
important because a great deal of body heat is lost through the head. Feet should be kept dry
and warm. Some people find it helpful to wear mittens and socks for bed during winter.
Chemical warmers, such as small heating pouches that can be placed in pockets, mittens,
boots, or shoes, can give added protection during long periods outdoors.Visit
www.raynauds.org.uk for information on useful recommended products. People who have
secondary Raynaud's should talk to their doctors before exercising outdoors in cold weather.
People with Raynaud's syndrome should also be aware that air conditioning can trigger
attacks. Turning down the air conditioning or wearing a sweater may help prevent attacks.
Some people find it helpful to use insulated drinking glasses and to put on gloves before
handling frozen or refrigerated foods.
Quit smoking
The nicotine in cigarettes causes the skin temperature to drop and the small arteries to
constrict which may lead to an attack.
Control stress
Because stress and emotional upsets may trigger an attack, particularly for people who have
primary Raynaud's, learning to recognise and avoid stressful situations may help control the
number of attacks. Many people have found that relaxation or biofeedback training can help
decrease the number and severity of attacks. Biofeedback training teaches people to bring the
temperature of their fingers under voluntary control. Local hospitals and other community
organisations, such as schools, often offer programmes in stress management.
Exercise
Many doctors encourage patients who have Raynaud's, particularly in the primary form, to
exercise regularly. Most people find that exercise promotes overall well-being, increases
energy level, helps control weight, and promotes restful sleep. Patients with Raynaud's should
talk to their doctors before starting an exercise programme.
See a doctor
People with Raynaud's should see their doctors if they are worried or frightened about attacks
or if they have questions about caring for themselves. They should always see their doctors if
attacks occur only on one side of the body (one hand or one foot) and any time if an attack
results in sores or ulcers on the fingers or toes.
Treatment With Medications
People with Raynaud's syndrome are more likely than those with primary Reynaud's
phenomenon to be treated with medications. Many doctors believe that the most effective and
safest drugs are calcium-channel blockers, which relax smooth muscle and dilate the small
blood vessels. These drugs decrease the frequency and severity of attacks in about two-thirds
of patients who have Raynaud's.
Some patients have found relief with drugs called alpha blockers that counteract the actions
of norepinephrine, a hormone that constricts blood vessels. Some doctors prescribe a
nonspecific vasodilator (drug that relaxes blood vessels), such as nitroglycerine paste or
patches. Patients should keep in mind that the treatment for Raynaud's is not always
successful. Often, patients with Raynaud's syndrome will not respond as well to treatment as
those with the primary form of the disorder.
Patients may find that one drug works better than another. Some people may experience side
effects that require stopping the medication. For other people, a drug may become less
effective over time. Women of childbearing age should know that the medications used to
treat Raynaud's may affect the growing foetus. Therefore, women who are pregnant or are
trying to become pregnant should avoid taking these medications if possible.
What about Reynaud's - is this different from Raynaud's?
Many people spell it in different ways but they are one and the same thing. The name
Raynaud’s was given to the condition by a French physician - Dr Maurice Raynaud in 1862.
Hence the name!
Contact details for self help organisation
For further information on Raynaud’s and scleroderma, patient support and advice, contact
the Raynaud’s & Scleroderma Association by visiting www.raynauds.org.uk, call 01270
872776 or email info@raynauds.org.uk

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Although the cause is unknown, several theories accoAlthough the cause is unknown, several
theories account for the reduced digital blood flow: intrinsic vascular wall hyperactivity to cold,
increased vasomotor tone due to sympathetic stimulation, and antigen-antibody immune response
(the most likely theory because abnormal immunologic test results accompany Raynaud’s
phenomenon). Risk factors include associated diseases (Buerger’s disease, atherosclerosis,
rheumatoid arthritis, scleroderma, and SLE) and smoking.

This disorder affects females more often than males.

unt for the reduced digital blood flow: intrinsic vascular wall hyperactivity to cold, increased vasomotor
tone due to sympathetic stimulation, and antigen-antibody immune response (the most likely theory
because abnormal immunologic test results accompany Raynaud’s phenomenon). Risk factors
include associated diseases (Buerger’s disease, atherosclerosis, rheumatoid arthritis, scleroderma,
and SLE) and smoking.

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 eMedicine Specialties > Rheumatology > Systemic Rheumatic Disease

Raynaud Phenomenon
Author: S Anita Narayanan, MD, Fellow in Rheumatology, University of Arizona College of Medicine
Coauthor(s): Jeffrey R Lisse, MD, FACP, Professor, Department of Internal Medicine, Chief, Section of
Rheumatology, University of Arizona School of Medicine; Mayra Oberto-Medina, DO, Fellow, Section of
Rheumatology, University of Arizona
Contributor Information and Disclosures
Updated: Jun 3, 2009

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Introduction

Background
Raynaud phenomenon manifests as recurrent vasospasm of the fingers and toes and usually occurs in
response to stress or cold exposure. The phenomenon is named for Maurice Raynaud, who, as a medical
student, defined the first case in 1862 as "episodic, symmetric, acral vasospasm characterized by pallor,
cyanosis, suffusion, and a sense of fullness or tautness, which may be painful."1

Secondary Raynaud phenomenon should be distinguished from primary Raynaud phenomenon (Raynaud
disease). They are distinct disorders that share a similar name. Raynaud disease is characterized by the
occurrence of the vasospasm alone, with no association with another illness. Secondary Raynaud
phenomenon is a designation usually used in the context of vasospasm associated with another illness,
most commonly an autoimmune disease.

There are several diagnostic criteria for primary Raynaud phenomenon, including attacks triggered by
exposure to cold and/or stress, symmetric bilateral involvement, an absence of necrosis, no detectable
underlying cause, normal capillaroscopy findings, normal laboratory findings for inflammation, and an
absence of antinuclear factors.2

Young female patients who have had Raynaud phenomenon alone for more than 2 years and have not
developed any additional manifestations are at low risk for developing an autoimmune disease. The same
should not be said for older patients and male patients with Raynaud phenomenon, as vasospastic
symptoms may predate systemic disease by as many as 20 years. In some studies, 46%-81% of affected
patients have secondary Raynaud phenomenon.

Although Raynaud phenomenon has been described with various autoimmune diseases, the most common
association is with progressive systemic sclerosis (90% in individuals with scleroderma) and mixed
connective-tissue disease (85% prevalence). Raynaud phenomenon has also been described with such
diverse diseases as systemic lupus erythematosus and other disorders not classified as autoimmune,
including frostbite, vibration injury, polyvinyl chloride exposure, and cryoglobulinemia.

Pathophysiology
In individuals with Raynaud phenomenon, one or more body parts experience intense vasospasm with
associated pallor and, often, cyanosis. This is often followed by a hyperemic phase with associated
erythema. The affected body parts are usually those most susceptible to cold injury. A clear line of
demarcation exists between the ischemic and unaffected areas. These effects are reversible, and they
must be distinguished from irreversible causes of ischemia such as vasculitis or thrombosis. Rarely, tissue
necrosis occurs distal to the affected vessel, usually in the periphery of the vasculature. It most commonly
affects the digits of the fingers but may affect the toes, nose, and ears. Occasionally, even the tongue is
involved.

Despite several years of research, the full understanding of the pathophysiology of Raynaud phenomenon
remains to be elucidated.

Primary Raynaud phenomenon is related to functional alterations alone. In contrast, secondary Raynaud
phenomenon also reflects structural microvascular abnormalities. Herrick (2005) reviewed the
pathogenesis of Raynaud phenomenon and describes the mechanisms under 3 categories: vascular,
neural, and intravascular abnormalities.3

Vascular abnormalities

• Endothelial dysfunction
○ A deficiency of vasodilatory mediators, including nitric oxide, has been implicated in the
pathogenesis of Raynaud phenomenon.4
○ Endothelin-1, a potent vasoconstrictor found in the endothelium, has been found to be
circulating in high levels in patients with secondary Raynaud phenomenon.5
 Release of endothelin-1 is triggered by vasoactive stimuli, including angiotensin,
vasopressin, and transforming growth factor-beta (TGF-beta).6
 A study by Rajagopalan et al (2003) reported increased levels of circulating
endothelin-1 in patients with secondary Raynaud phenomenon.4
 There have been conflicting results regarding the levels of endothelin-1 in
patients with primary Raynaud phenomenon.
○ Angiotensin also has vasoconstrictive and profibrotic effects.7

 A 2004 study by Kawaguchi et al revealed higher levels of angiotensin II in

patients with diffuse cutaneous systemic sclerosis.8
 Based on these results, treatment with angiotensin-converting enzyme (ACE)
inhibitors needs further investigation.
• Structural abnormalities
○ In patients with systemic sclerosis, Raynaud phenomenon differs from primary Raynaud
disease.9
 ○ It is related to fibrotic proliferation of the vasculature leading to reduced blood flow to the
digits.
Neural abnormalities

• Central mechanisms
○ Edwards et al (1998) performed a series of experiments showing that patients with
primary Raynaud phenomenon do not habituate to stressful stimuli in the same way as
healthy control subjects. The ability to habituate is described as vasodilation in forearm
muscles and vasoconstriction in the cutaneous circulation of skin.
○ Based on these data, it is presumed that patients with Raynaud phenomenon repeatedly
undergo cutaneous vasoconstriction to many stressful stimuli. Healthy individuals are able
to habituate, thus not displaying these responses.10
• Impaired vasodilation
○ An important neuropeptide, calcitonin gene-related peptide, is a potent vasodilator
secreted by nerves that supply blood vessels.11
○ A diminished number of calcitonin gene-related peptide–releasing neurons has been
found in skin biopsy samples of patients with primary Raynaud and systemic sclerosis.12
○ Neuropeptide Y, a potent vasoconstrictor, has been found in increased levels in Raynaud
phenomenon secondary to systemic sclerosis.
• Impaired vasoconstriction
○ α2C -adrenoreceptors overactivity: α2C -adrenoreceptors have been found to enable cold-
induced vasoconstriction of the blood vessels.13
○ Two studies by Furspan et alshowed that the enhanced contractile response to α2
-adrenergic agonists and cooling in patients with primary Raynaud phenomenon may be
linked to increased protein tyrosine kinase activity.14
○ These data provide information regarding the possible benefits of protein tyrosine kinase
inhibitors in the treatment of Raynaud phenomenon.
Intravascular abnormalities

• In primary Raynaud and systemic sclerosis, increased platelet activation and aggregation has
been demonstrated.15
• An increased production of platelet thromboxane A2, a potent vasoconstrictor, has been found in
patients with Raynaud phenomenon.
• In patients with systemic sclerosis, an impaired fibrolytic system has been reported, probably
contributing to vascular obstruction.16
• Oxidative stress by reactive oxygen species has also been implicated in the pathogenesis of
Raynaud phenomenon.
Frequency
United States

• A 7-year study of Raynaud phenomenon in whites in the United States showed baseline
prevalence rates of 11% in women and 8% in men and yearly incidence rates of 2.2% in women
and 1.5% in men.17
International
 • The prevalence of primary Raynaud phenomenon varies among different populations, from 4.9%-
20.1% in women to 3.8%-13.5% in men.
• As in the United States, the prevalence of secondary Raynaud phenomenon depends on the
underlying disorder.
Mortality/Morbidity
• Primary Raynaud phenomenon does not usually cause death or serious morbidity. However, in
very rare cases, ischemia of the affected body part can result in necrosis.
• Secondary Raynaud phenomenon is important as a possible marker for other diseases that may
lead to morbidity and mortality. Examples of this include scleroderma (progressive systemic
sclerosis), systemic lupus erythematosus, and hyperviscosity syndromes.
Race
• Primary Raynaud phenomenon has no racial predilection.
• Secondary Raynaud phenomenon approximates the racial prevalence of the underlying disease, if
any.
Sex
• The prevalence of primary Raynaud phenomenon varies in different populations, ranging from
4.9%-20.1% in women to 3.8%-13.5% in men.
Age
• Primary Raynaud phenomenon usually occurs in the second or third decade of life.
• Secondary Raynaud phenomenon begins in accordance with the underlying disorder.
Clinical

History
• Numbness and pain in the affected area or areas may be present.
• Affected areas show at least two color changes: white (pallor), blue (cyanosis), and red
(hyperemia).
○ The color changes are usually in the order noted, but not always.
○ These changes should be reversible but may, in severe cases, lead to local ischemia and
ulceration.
• Any history of associated symptoms should raise suspicion of an underlying disorder. History of
other vasospastic symptoms such as migraines may be useful.
• Obtain occupational history.
○ Secondary Raynaud phenomenon has been associated with the frequent use of vibrating
tools such as jackhammers and sanders.
 Photo of a patient with Raynaud phenomenon that resulted from working
with a jackhammer. Courtesy of the CDC.

[ CLOSE WINDOW ]

Photo of a patient with Raynaud phenomenon that resulted from working

with a jackhammer. Courtesy of the CDC.

○ Industrial exposure to polyvinyl chloride has been implicated.

○ Any history of injury or frostbite may leave the involved limb vulnerable to vasospasm.

• Syndromes associated with Raynaud phenomenon include the following:

○ Autoimmune disorders

 Progressive systemic sclerosis (scleroderma) including the diffuse and limited

(formerly called CREST syndrome)
 Systemic lupus erythematosus
 Mixed connective-tissue disease (and other overlap syndromes)
 Dermatomyositis and polymyositis
 Rheumatoid arthritis
 Sjögren syndrome
 Vasculitis
  Primary pulmonary hypertension
○ Infectious syndromes

 Hepatitis B and C infections (especially associated with mixed or type 3

cryoglobulinemia)
 Mycoplasma infections (with cold agglutinins)
○ Neoplastic syndromes

 Lymphoma
 Leukemia
 Myeloma
 Waldenström macroglobulinemia
 Polycythemia
 Monoclonal or type 1 cryoglobulinemia
 Lung adenocarcinoma
 Other paraneoplastic disorders
○ Environmental associations

 Vibration injury
 Vinyl chloride exposure
 Frostbite
 Lead exposure
 Arsenic exposure
○ Metabolic/endocrine syndromes

 Acromegaly
 Myxedema
 Diabetes mellitus
 Pheochromocytoma
 Fabry disease
○ Hematologic syndromes

 Paroxysmal nocturnal hemoglobinuria

 Polycythemia
 Cryofibrinogenemia
○ Drug-related associations

 Oral contraceptives
 Ergot alkaloids
 Bromocriptine
 Beta-adrenergic blocking drugs
 Antineoplastics (eg, vinca alkaloids, bleomycin, cisplatin)
 Cyclosporine
 Alfa-interferon
 • Syndromes that may be confused with Raynaud phenomenon are as follows:
○ Anatomic syndromes

 Carpal tunnel syndrome

 Reflex sympathetic dystrophy syndromes
 Thoracic outlet syndrome
○ Miscellaneous circulatory syndromes

 Atherosclerosis
 Thromboangiitis obliterans
 Vasculitis
 Thromboembolic disease
○ Vasospastic syndromes

 Livedo reticularis
 Acrocyanosis
 Chilblains
Physical
• Carefully examine digits if either primary or secondary Raynaud is suspected.
○ Observe for sclerodactyly, calcinosis, or digital ulcers.
○ Examine nailfold capillaries under magnification from a dissecting microscope or
ophthalmoscope to help diagnose underlying autoimmune disorders.
○ Abnormalities often appear in patients with early scleroderma. The normally regular
pattern of capillary loops is replaced with abnormally large loops, alternating with areas
without any capillaries.
• Evaluate any signs or symptoms of other syndromes associated with secondary Raynaud.
○ Bone pain may suggest a paraneoplastic syndrome associated with a hyperviscosity
syndrome.
○ The presence of nephritis, malar erythema, and arthritis suggests systemic lupus
erythematosus.
• Persistent cyanosis or necrotic distal tissue suggests an underlying disorder or permanent
ischemia. Livedo reticularis suggests an autoimmune disorder or coagulation abnormality.
• Carpal tunnel syndrome has been associated with an increased frequency of Raynaud
phenomenon.
Causes
• The cause of primary Raynaud phenomenon remains unknown.
• Possible causes for secondary Raynaud can be divided into several broad categories, including
the following:
○ Occupational
○ Hematologic
○ Collagen-vascular (autoimmune)
○ Medication-induced
 ○ Miscellaneous syndromes such as Fabry disease, pheochromocytoma, lung
adenocarcinoma, acromegaly, carpal tunnel syndrome, and myxedema
• Although the following entities do not usually have the same inciting causes, nor do they
encompass the usual color changes associated with Raynaud phenomenon, they can easily be
mistaken for Raynaud phenomenon:
○ Vasculitis
○ Carpal tunnel syndrome
○ Reflex sympathetic dystrophy
○ Thromboembolic disease
○ Thoracic outlet syndrome

More on Raynaud Phenomenon

Overview: Raynaud Phenomenon

Differential Diagnoses & Workup: Raynaud Phenomenon

Treatment & Medication: Raynaud Phenomenon

Follow-up: Raynaud Phenomenon

Multimedia: Raynaud Phenomenon

References

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References

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[ CLOSE WINDOW ]

Further Reading
[ CLOSE WINDOW ]
Keywords

Raynaud's phenomenon, Raynaud phenomenon, vasospasm, Raynaud disease, Raynaud’s disease,

reversible ischemia of peripheral arterioles, exposure to cold, stress-related ischemia, systemic sclerosis,
scleroderma, secondary Raynaud, secondary Raynaud's, vasospasm, pallor, white finger disease
[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

S Anita Narayanan, MD, Fellow in Rheumatology, University of Arizona College of Medicine

S Anita Narayanan, MD is a member of the following medical societies: American College of Physicians,
American College of Rheumatology, and American Medical Association
Disclosure: Nothing to disclose.
Coauthor(s)

Jeffrey R Lisse, MD, FACP, Professor, Department of Internal Medicine, Chief, Section of Rheumatology,
University of Arizona School of Medicine
Jeffrey R Lisse, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American
College of Physicians-American Society of Internal Medicine, American College of Rheumatology,
American Geriatrics Society, and Sigma Xi
Disclosure: Nothing to disclose.
 Mayra Oberto-Medina, DO, Fellow, Section of Rheumatology, University of Arizona
Disclosure: Nothing to disclose.
Medical Editor

John Varga, MD, Professor, Department of Internal Medicine, Division of Rheumatology, Northwestern
University
John Varga, MD is a member of the following medical societies: American College of Physicians, American
College of Rheumatology, Central Society for Clinical Research, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.
Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.
Managing Editor

Elliot Goldberg, MD, Dean of the Western Pennsylvania Clinical Campus, Professor, Department of
Medicine, Temple University School of Medicine
Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College
of Physicians, and American College of Rheumatology
Disclosure: Nothing to disclose.
CME Editor

Alex J Mechaber, MD, FACP, Associate Dean for Undergraduate Medical Education, Associate Professor
of Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha,
American College of Physicians-American Society of Internal Medicine, and Society of General Internal
Medicine
Disclosure: Nothing to disclose.
Chief Editor

Herbert S Diamond, MD, Professor of Medicine, Temple University School of Medicine; Chairman
Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital
Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American
College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta
Kappa
Disclosure: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; West
Penn Allegheny Health System None Board membership

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