raynauds phenomenon-dignosis & evaluation
TRANSCRIPT
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)