Raynaud's phenomenon is characterized by episodic vasospasm and ischemia of the extremities in response to cold or stress. Attacks typically involve color changes from white to blue to red. Primary Raynaud's has no known cause, while secondary Raynaud's is associated with underlying conditions like scleroderma. Nifedipine is currently the only drug approved for treatment, though research continues on therapies like topical nitroglycerin and rho kinase inhibitors to promote vasodilation.

1. DR.W.A.P.S.R.WEERARATHNA
REGISTRAR – WARD 10/02

2.  What is Raynaud’s phenomenon
 Classificatin/types
 Raynaud’s phenomenon vs Acral cyanosis
 Pathogenesis of Raynaud’s phenomenon
 Clinical presentation
 Diagnostic work-up/evaluation of a patient
Treatment/management
Summary
Refferences

4. Episodic digital ischemia manifested
clinically by the sequential development of
digital blanching ,cyanosis, and rubor of
the fingers/toes after cold exposure &
subsequent rewarming.

5.  Primary Raynaud’s / Raynaud’s disease
the causes is not known.(idiopathic)
Secondary Raynaud’s / Raynaud’s
phenomenon where the causes are
known.

6. Expose to cold /
triggering factor
Digital arteries at
fingers and toes
vasospasm
Become pale, less
blood flow and low
O2 supply
Capillaries/venules
dialate
Cyanosis due to
deoxygenate blood
Rewarming-
(arteries dilate)
Blood flow increase,
high O2 supply
Reactive
hyperemia- Color
change to bright
red
Affected area is
warm and
throbbing pain

10.  Acrocyansis-
 Persistent, painless, symmetric cyanosis of
the hands, feet, or face caused by
vasospasm of the small vessels of the skin in
response to cold.
 The digits and hands or feet are persistently
cold and bluish, sweat profusely, and may
swell.
 Unlike Raynaud syndrome, cyanosis persists
and is not easily reversed, trophic changes
and ulcers do not occur, and pain is absent.
Pulses are normal.

11. 1.Primary or idiopathic Raynaud’s
phenomenon : Raynaud’s disease
2. secondary Raynaud’s phenomenon :
 1. Collagen vascular disease-
Scleroderma
SLE
RA
DM
PM

12.  2. Arterial occlusive disease
ATH of the extremities
Thromboangitis obliterans
Acute arterial occlusion
Thorasic outlet syndrome
3. Pulmonary hypertension
4. Neurologic disorders
Intervertebral disc disease
Syringomyelia
Spinal cord tumour
Stroke
PM
CTS

13. 5. Blood dyscrasias
Cold agglutinins
Cryoglobulinemias
Cryofibrogenemias
Myeloproliferative disorders
Waldenstrom’s
macroglobulinemia

14.  6. Trauma
Vibration injury
Hammer hand syndrome
Electric shock
Cold injury
Typing
7. Drugs
Ergot derivatives
Methyl sergide
BB
Bleomycin
Vinblastin
Cisplatin

15. Over 50% of patients with Raynaud’s
phenomeneon
Male:female = 1:5
Age- between 20 & 40 years
Figers > Toes
 One or 2 finger tipsentire fingerall
fingers in subsequent attacks
Rarely ear lobes/tip of the nose/penis!

16. Occurs in frequently with migrain
headaches & varient angina vasospstic
disorders!
Physical exam- entirely normal
Fingers & toes may be cool between
attacks
May perspire excessively
Sclerodactyly in about 10%
Angiography of digits not indicated
Milder phenomenon-<1% loose a part of a
digit
Spontaneous improvement in 15%
Progressive disease in 30%

17. Ssc- about 80-90% have the disease
 presenting symptom in 30%
 Ischaemic
fingertipulcersgangreneautoamputation
SLE- 20% have the disease
DM/PM- 30% of patients
RA- frequently occurs
 Arteriosclerosis of the extremities-men
>50 years
 Burger’s disease-uncommon,young,smoking
men

18. Large/medium sized arterial occlusion
due to thrombus
Thorasic outlet syndrome- diminished
intravascular pressure/ sympathetic
stimulation in brachial plexus
PHT-neurohormonal abnormalities in both
pulmonary & digital arteries
Blood dyscrasias-precipitation of plasma
proteins/huperviscosity/RBS & PLT
aggregation

19. Raynaud phenomenon can be diagnosed
on clinical grounds.
Imaging studies, including thermography,
isotope studies, and arteriography, have all
been used, but none has proven superior
to clinical assessment.
 However, patients with a fixed,
nonreversible, cyanotic lesion require
further evaluation of the vasculature.

20.  FBC with indices - To evaluate for polycythemic
disorders, underlying malignancies, or autoimmune
disorders
 RFT/BUN - To evaluate for possible renal
impairment or dehydration
 S.Creatinine - To evaluate for possible renal
impairment
 PT/INR - To observe for any evidence of hepatic
dysfunction
 APTT - To observe for any evidence of
antiphospholipid antibody disorder or hepatic
dysfunction
 Serum glucose - To evaluate for diabetes
 TFT - To test for thyroid disorders

21.  ANA - May be positive in autoimmune disorders
and should be obtained in patients with features of
these disorders
 Serum viscosity - Elevated in hyperviscosity
syndromes such as paraproteinemias
 Serum CPK- Elevated in muscle damage such as
PM/DM
 RF - May be elevated in RA, other autoimmune
disorders, and some forms of cryoglobulinemia
(monoclonal proteins in MM and Waldenström
macroglobulinemia have an increased frequency of
rheumatoid factor activity)

22.  Hepatitis panel - Positive for HBV/HCV infection in
many patients with cryoglobulinemia
 Cold agglutinins - Present in Mycoplasma
infections and lymphomas
 Heavy metal screen - To asses for neuropathic
pain due to poisoning
 Growth hormone - To evaluate for acromegaly
 Plasma metanephrine testing or 24-hour urinary
collection for catecholamines and metanephrines -
To evaluate for pheochromocytoma
 LAP score - To evaluate for leukemias in
appropriate patients

23. Antiphospholipid antibodies studies -
Including dilute Russell viper venom
studies, anticardiolipin antibodies, and
anti-beta-1-glycoprotein-2 antibodies
Serum protein and urine electrophoresis -
To evaluate for paraproteinemias
Flow cytometry or acidified serum lysis
(Ham) test - To evaluate for PNH

26.  Nondrug therapy may be all that is required for
mild cases of primary Raynaud phenomenon.
 With time, most patients learn to incorporate these
therapies on their own.
 Avoiding inciting environmental factors, such as
direct contact with frozen foods or cold drinks
 Insulation against cold and local warming,
including gloves or heavy socks and electric and
chemical warming devices
 Discontinuing drugs that may provoke vasospasm
 Avoiding smoking

27. Laser therapy may result in less frequent,
less severe attacks. (This therapy needs
more studies!)
Studies of acupuncture have been limited,
but have suggested some benefit.
 Biofeedback and relaxation have shown
no difference in frequency or severity of
attacks.

28.  CCB’s- the class of drugs most widely used
for treatment of Raynaud syndrome—
especially the dihydropyridines, the most
potent vasodilators.
 Nifedipine is the customary first choice. The
usual dosage is 30-120 mg of the extended-release
formulation taken once daily.
 Start with the lowest dose and titrate up as
tolerated.
 If adverse effects occur, decrease the dosage
or use another agent, such as nicardipine, or
a non-dihydropyridine calcium channel
blocker such as such as amlodipine or
diltiazem.

29. Patients should check their blood pressure
regularly and may want to keep a log of
the number and severity of attacks.
This may help in evaluating the efficacy of
therapeutic management.
Other medications that have been studied
in Raynaud phenomenon include the
following:

30. Topical nitroglycerin (1% or 2%)
 Iloprost (prostaglandin analog)
 Selective serotonin reuptake inhibitors
(SSRIs)
Phosphodiesterase-5 enzyme inhibitors
(sildenafil, tadalafil, vardenafil)
 Losartan
 Bosentan (endothelin receptor antagonist) –
Orphan drug for treating new digital ulcers in
patients with systemic sclerosis
 Botulinum toxin
N-acetylcysteine – In patients with systemic
sclerosis and digital ulcers

31. Therapy with antiplatelet agents has been
attempted but has not been proved
effective.
 RCT by Gliddon et al showed no
significant difference in attack frequency
or severity between the ACEI quinapril and
placebo.
High-quality, well-designed, RCT’s are
needed to study the effect of other
pharmacotherapy.
 Anticoagulation is not indicated, except in
rare cases of rapidly advancing digital
ischemia.

32. Rho kinase inhibitors
• Responsible for cold-induced expression of alpha-
2 adrenoceptors/vasodialators.
Statins
• In part due to Rho kinase inhibition
Antiplatelet treatments?
• Current trial at RNHRD (for primary and
secondary Raynaud’s)

33.  Raynaud’s phenomenon is caused by episodic
vasospasm and ischaemia of the extremities,
particularly the digits, in response to cold or emotional
stimuli
 Attacks comprise a colour change in extremities from
white (ischaemia), to blue (deoxygenation), and then to
red (reperfusion)
 Primary Raynaud’s phenomenon is an exaggerated
response to stimuli, with no known underlying cause
 Secondary Raynaud’s phenomenon is usually caused
by connective tissue disease and patients are more
likely to develop tissue damage
 Nifedipine is currently the only drug licensed for use in
Raynaud’s phenomenon
 Key areas of ongoing research include a topical
nitroglycerin and a rho kinase inhibitor (vasodilator)