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Raynaud's Phenomenon
Aka: Raynaud's Phenomenon, Raynaud's Syndrome, Raynaud Disease, Raynaud Phenomenon, Raynaud Syndrome- See Also
- Thromboangiitis Obliterans (Buerger's Disease)
- CREST Syndrome
- Epidemiology
- Affects 3-4% of U.S. adult population
- Predominately affects women by 3 to 1 ratio
- Onset from Puberty to age 30 years
- Family History responsible in 20-30% of patients
- Pathophysiology
- Exaggerated response to cold Temperatures
- Increased Alpha Adrenergic Receptor responsiveness
- May be primary idiopathic or secondary cause
- Causes: Secondary Causes of Raynaud's Phenomenon
- Connective Tissue Disease
- Scleroderma (95% have Raynaud's)
- Systemic Lupus Erythematosus
- Sjogren's Syndrome
- Dermatomyositis
- Trauma
- Occupational tool use (vibratory tool)
- Carpal Tunnel Syndrome
- Occlusive vascular disease
- Atherosclerosis
- Systemic Vasculitis
- Thromboembolism
- Thromboangiitis Obliterans (Buerger's Disease)
- Medications
- See provocative factors below
- Hyperviscosity state (e.g. Polycythemia Vera)
- Paraproteinemia
- Cryoglobulinemia
- Connective Tissue Disease
- Causes: Provocative Factors
- Tobacco
- Caffeine
- Amphetamines
- Cocaine
- Pseudoephedrine
- Phenylpropanolamine
- Ephedrine
- Phenylephrine
- Ergotamines
- Triptans
- Phentermine (Qsymia)
- Unopposed Estrogen
- Nonselective Beta Blockers
- Clonidine
- Chemotherapeutic medications (e.g. Bleomycin)
- Symptoms
- Hypersensitivity to cold Temperatures
- Color changes of digits during cold or stress exposure
- "White attacks" suggest severe ischemia
- Mottling with acrocyanosis is more common and benign
- Sensation of numbness, clumsiness or "pins and needles"
- One finger may be more sensitive than the others
- Signs
- Pallor or Cyanosis of fingers or toes
- Thumb is not involved
- Evaluation
- Distal pulses
- Bruit Auscultation
- Signs of ischemia
- Allen's Test
- Assess for Thoracic Outlet Syndrome
- Labs
- Complete Blood Count (CBC)
- Serum Chemistry Panel (Chem7)
- Urinalysis
- Consider Antinuclear Antibody (ANA) when indicated
- Imaging
- Consider Arterial Doppler Ultrasound
- Diagnosis: Cold Challenge (not necessary)
- Immerse patient's hand in ice water
- Blanching occurs in seconds
- Next Cyanosis occurs
- Rewarm hand in warm water
- Erythema and pain may occur on rewarming
- Immerse patient's hand in ice water
- Complications
- Cutaneous ulceration of involved digit
- Gangrene
- Management: First Line
- Conservative Measures
- Smoking Cessation
- Avoid precipitating medications (see above)
- Limit Caffeine
- Avoid cold or reduce cold exposure
- Dress warmly in loose-fitting layers for the cold
- Wear a warm hat
- Wear mittens instead of gloves, and wear stockings
- Use hand warmers (chemical heat packets)
- Use a space heater at work
- Preheat car during winter
- Calcium Channel Blockers (Dihydropyridines)
- Nifedipine ER (Procardia) orally daily
- Amlodipine
- Other medications
- Sildenafil (Viagra)
- Nitroglycerin Ointment (Nitro-Bid 2%)
- Apply to affected fingers or toes four times daily
- Do not use with Sildenafil (risk of Hypotension)
- Sodium Nitrate with Ascorbic Acid gel
- Conservative Measures
- Management: Severe Ischemia (e.g. CREST Syndrome related)
- See first-line measures above
- Consider Thromboangiitis Obliterans (Buerger's Disease)
- Alpha-adrenergic blockers
- Angiotensin Receptor Blockers
- Pentoxifylline (Trental)
- Management: Finger Temperature Feedback
- One Protocol
- Patient places fingertip on Temperature monitor
- Tone played louder when finger gets colder
- Patient tries to warm finger to decrease noise
- Reduces symptoms by 92%
- Second Protocol
- Patient immerses hands in warm water
- Rest of patient's body cold (e.g. outside)
- Repeat tid, every other day, for 3 weeks
- New conditioned cold response: Vasodilation
- One Protocol
- Management: Severe or ischemic digital ulcers
- Intravenous Prostaglandins
- PGI2 Analog: Iloprost (not available in U.S.)
- PGI2: Epoprostenol
- Cervical sympathectomy
- Proximal sympathectomy
- Localized microsurgical digital sympathectomy
- Local chemical sympathectomy with Lidocaine
- Intravenous Prostaglandins
- References
- (2018) Presc Lett 25(12): 70
- Oreizi-Esfahani (1996) Consultant, p. 905-12
- Wigley (1999) Consultant p. 540-54
- Comfort-Adee (1993) Am Fam Physician, 47(4): 823-9 [PubMed]