Raynaud syndrome, also known as Raynaud's phenomenon, is a medical condition in which spasm of arteries cause episodes of reduced blood flow

1. by : ramin mohammadi

2. INTRODUCTION
a medical condition in which spasm of arteries cause
episodes of reduced blood flow. Typically, the fingers, and
less commonly the toes, are involved. Rarely, the nose,
ears, or lips are affected.

3. 1. RP is considered primary if these symptoms occur
alone without evidence of any associated disorder
2. secondary RP refers to the presence of the disorder in
association with a related illness, such as systemic
lupus erythematosus and systemic sclerosis (SSc;
scleroderma)

4. EPIDEMIOLOGY
 estimates of the prevalence of
RP have ranged from 3 to 20
percent in women and 3 to
14 percent in men.
 RP is more common among
young women, younger age
groups, and family
members of patients with RP

5. Primary Raynaud phenomenon
 is term to describe those patients without a
definable cause for their vascular events
 usually has an age of onset between 15 and 30 years of
age, is more common in women, and may occur in
multiple family members
 while primary RP often begins in the teenage years,
careful assessment for secondary causes is most
important

6.  There is a reported increase in prevalence of RP among
patients with fibromyalgia syndrome
 Similarly, there are several surveys that support an
association of migraine headaches and RP

7. Secondary Raynaud phenomenon
 Secondary RP refers to those patients with RP in
whom an associated disease or cause may underlie the
attacks:
 Diseases commonly associated with RP include
autoimmune rheumatic diseases such as systemic
sclerosis (SSc; scleroderma), systemic lupus
erythematosus, mixed connective tissue disease,
Sjögren's syndrome and
dermatomyositis/polymyositis.

8. Various drugs or toxins can also
precipitate or exacerbate RP such
as amphetamines and
chemotherapeutic agents
(especially cisplatin and
bleomycin)
Occupational and environmental
causes of RP include vascular
trauma (eg,injury to the distal
ulnar artery in the hypothenar
hammer syndrome) the use of
vibrating tools frostbite and
carpal tunnel syndrome

9.  Hypothyroidism may predispose to RP, and
improvement of cold induced vasospasm may occur
with thyroid hormone replacement. Vasculitis may
cause ischemic digits, but the absence of preceding
cold-induced and reversible color changes help
distinguish this from true RP.

10. CLINICAL MANIFESTATIONS
With both primary and secondary RP, a typical episode
is characterized by the sudden onset of cold fingers
(or toes) in association with sharply demarcated color
changes of skin pallor (white attack) due to constricted
blood flow, followed by cyanotic skin (blue attack)
which indicates tissue hypoxia
With rewarming, the ischemic phase (white or blue
attack) usually lasts for 15 to 20 minutes. The skin
subsequently blushes upon recovery, thereby resulting
in the erythema of reperfusion.

12.  A Raynaud attack typically begins in a single finger
and then spreads to other digits symmetrically in
both hands
 The index, middle, and ring finger are the most
frequently involved digits, while the thumb is often
spared entirely.
 so , Involvement of the thumb may indicate a
secondary cause of RP

13. Livedo reticularis
 During a cold response, patients with RP may exhibit
livedo reticularis; this is a violaceous mottling or
reticular pattern of the skin of the arms and legs,
sometimes with regular unbroken circles. In those
with primary RP, this finding is benign and completely
reversible with rewarming

14.  By comparison, nonreversible livedo reticularis may
be observed in patients with vasculitis, occlusive
vascular disease (eg, due to atheroemboli or
thrombosis), or antiphospholipid syndrome.
These cutaneous changes may also be associated with
irregular broken circles.

15. EVALUATION AND DIAGNOSIS
Our evaluation begins with the following three
screening questions for RP :
● Are your fingers unusually sensitive to cold?
●Do your fingers change color when they are exposed to
cold temperatures?
●Do your fingers turn white, blue, or both?
We diagnose RP if the patient has a positive response to
all three questions

16. Nailfold capillary microscopy
Nailfold capillary microscopy is
performed by dropping oil on
the periungual area and
examining with an
ophthalmoscope set at diopter
40 or with a dissecting
microscope.
Nailfold capillaroscopy is
the method most
commonly used in
clinical practice to help
distinguish patients with
primary RP from those
with secondary RP.

17. Enlarged or distorted capillary loops and/or
dropout or loss of loops suggest an underlying
(or an increased likelihood of developing)
autoimmune rheumatic disease
If the enlargement is associated with loss of
capillaries, then the patient is more likely to have
or develop SSc

18.  Every patient with a diagnosis of RP should be
carefully evaluated to distinguish primary RP from the
secondary disorder.
 We diagnose primary RP if the results of a thorough
history and physical examination, including nailfold
capillary microscopy, do not suggest secondary RP.
There is typically no need for further specialized
testing among such patients

19. Patients with a moderate or high clinical suspicion of a
secondary cause of RP may have the following clinical
features:
• Later age of onset (greater than 40 years)
• History of a known precipitant
• Male gender
• Painful severe events with tissue sign of ischemia (ulceration)
• Asymmetric attacks
• RP associated with signs or symptoms of another disease
• Abnormal nailfold capillaries
• Abnormal laboratory parameters suggesting vascular disease or an
autoimmune disorder
•RP associated with ischemic signs or symptoms proximal to the
fingers (such as the hand or arm) or toes (foot or limb)

20.  There are several conditions and disorders that can mimic
RP including:
excessive cold sensitivity
external compression of blood vessels
peripheral neuropathy
complex regional pain syndrome
occlusive vascular disease
acrocyanosis
acute idiopathic blue finger
erythromelalgia

21. Initial treatment of the Raynaud phenomenon
 The goals of therapy in patients with the Raynaud
phenomenon (RP) are to improve quality of life and to
prevent ischemic tissue injury. At least a moderate
reduction in the intensity of attacks and the
prevention of digital ulcers or tissue injury are
achievable in most patients. However, abolishing cold
sensitivity and eliminating all Raynaud events is not
likely with available treatment options, particularly in
patients with secondary RP

22.  We advise that all patients should be educated
regarding both the potential causes of a Raynaud
attack and the general measures to help prevent and
terminate an episode. Patients with primary RP are
unlikely to develop damaging digital ischemia and
thus are considered good candidates for
nonpharmacologic therapies

23. General measures that help to prevent or diminish the
severity of attacks of RP include:
●Avoidance of cold exposure, especially sudden changes
such as walking into the frozen food section of a grocery
store.
●Use of strategies to keep the whole body warm,
including dressing warmly (eg,with thermal underwear,
layered clothing, and a heat-conserving hat).
●Use of strategies to keep the digits of the hands and
feet warm (eg, winteer gloves, chemical hand warmers,
and heavy wool stockings).
●Knowledge of methods to help terminate an attack of RP.
These include placing the hands under warm water or
in a warm place (such as the axilla) or rotating arms in a
whirling or windmill pattern. Rubbing the hands
together can help.

24. ● Avoidance of rapidly changing temperatures, such as occurs when
quickly moving from a hot environment (90ºF) into an air-conditioned
room (70ºF).
● Avoiding sitting motionless in cool breezes or in humid cold air is
also recommended.
● Avoidance of smoking is advised since regular smokers are sensitized to
the vasoconstrictive properties of cigarettes.
●Avoidance of sympathomimetic drugs (such as decongestants,
amphetamines, diet pills, herbs containing ephedra), which is generally
recommended, although studies evaluating the degree of the impact of
over-the-counter preparations (such as cold medications) have not been
performed .
● Avoidance of agents used to treat attention deficit hyperactivity disorder
(methylphenidate and dextroamphetamine) is recommended.

25. ●Avoidance of some of the medications used for migraine
headaches, including serotonin agonists (eg, sumatriptan) or
caffeine plus ergotamine.
● Avoidance of repeated trauma to the fingertips by all
patients with RP and avoidance of vibrating tools by patients
with vibration-induced RP .
● Control or limitation of emotional stress, because the
thermoregulatory vessels are constricted by increased
sympathetic tone. Stress plus cold exposure is an especially
potent trigger for RP

26. Pharmacotherapy
 We recommend use of the slow-release or long-acting
preparations of the dihydropyridine calcium channel
blockers (CCBs)
 Effective doses of nifedipine range from 30 to 180
mg/day, and effective doses of amlodipine range
from 5 to 20 mg/day
 start with the lowest dose and gradually increase, if
needed, depending upon the response
 Some experts feel that amlodipine may be better
tolerated in patients with low blood pressure at
baseline

27.  The major side effects associated with the dihydropyridines
include headache, dizziness, flushing, tachycardia,
and edema.
 Maximal doses of the calcium channel blocker (eg,
amlodipine 15 to 20 mg/day) may lead to significant
edema
 Alternative therapies may be required in patients in whom
CCBs are contraindicated or are poorly tolerated at low
doses (eg, patients with severe gastrointestinal
dysmotility, severe pulmonary artery hypertension,
significant cardiac disease with edema, and low blood
pressure).

28. ● A phosphodiesterase-5 inhibitor, such as sildenafil
● Topical nitrates
●An alternative oral agent, such as losartan or fluoxetine
● Local injection of botulinum toxin type A is also an
option