DR.W.A.P.S.R.WEERARATHNA

REGISTRAR – WARD 10/02

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

Episodic digital ischemia manifested

clinically by the sequential development of

digital blanching ,cyanosis, and rubor of

the fingers/toes after cold exposure &

subsequent rewarming.

Primary Raynaud’s / Raynaud’s disease

the causes is not known.(idiopathic)

Secondary Raynaud’s / Raynaud’s

phenomenon where the causes are

known.

Expose to cold / triggering factor

Digital arteries at fingers and toes

vasospasm

Become pale, less blood flow and low

O2 supply

Capillaries/venulesdialate

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

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.

1.Primary or idiopathic Raynaud’s

phenomenon : Raynaud’s disease

2. secondary Raynaud’s phenomenon :

1. Collagen vascular disease-

Scleroderma

SLE

RA

DM

PM

2. Arterial occlusive diseaseATH of the extremitiesThromboangitis obliteransAcute arterial occlusionThorasic outlet syndrome

3. Pulmonary hypertension4. Neurologic disorders

Intervertebral disc diseaseSyringomyeliaSpinal cord tumourStrokePMCTS

5. Blood dyscrasias

Cold agglutinins

Cryoglobulinemias

Cryofibrogenemias

Myeloproliferative disorders

Waldenstrom’s

macroglobulinemia

6. TraumaVibration injuryHammer hand syndromeElectric shockCold injuryTyping

7. DrugsErgot derivativesMethyl sergideBBBleomycinVinblastinCisplatin

Over 50% of patients with Raynaud’s

phenomeneon

Male:female = 1:5

Age- between 20 & 40 years

Figers > Toes

One or 2 finger tipsentire fingerall

fingers in subsequent attacks

Rarely ear lobes/tip of the nose/penis!

Occurs in frequently with migrainheadaches & varient angina vasospsticdisorders!

Physical exam- entirely normalFingers & toes may be cool between

attacksMay perspire excessivelySclerodactyly in about 10%Angiography of digits not indicatedMilder phenomenon-<1% loose a part of a

digitSpontaneous improvement in 15%Progressive disease in 30%

Ssc- about 80-90% have the diseasepresenting symptom in 30% Ischaemic

fingertipulcersgangreneautoamputationSLE- 20% have the diseaseDM/PM- 30% of patientsRA- frequently occursArteriosclerosis of the extremities-men

>50 yearsBurger’s disease-uncommon,young,smoking

men

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

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.

FBC with indices - To evaluate for polycythemicdisorders, 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

ANA - May be positive in autoimmune disorders and should be obtained in patients with features of these disorders

Serum viscosity - Elevated in hyperviscositysyndromes 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 Waldenströmmacroglobulinemia have an increased frequency of rheumatoid factor activity)

Hepatitis panel - Positive for HBV/HCV infection in many patients with cryoglobulinemia

Cold agglutinins - Present in Mycoplasmainfections 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

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

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

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.

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.

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:

Topical nitroglycerin (1% or 2%) Iloprost (prostaglandin analog)Selective serotonin reuptake inhibitors

(SSRIs)Phosphodiesterase-5 enzyme inhibitors

(sildenafil, tadalafil, vardenafil)LosartanBosentan (endothelin receptor antagonist) –

Orphan drug for treating new digital ulcers in patients with systemic sclerosis

Botulinum toxinN-acetylcysteine – In patients with systemic

sclerosis and digital ulcers

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.

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)

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)