Raynaud's phenomenon

see also:

• rheumatology

• recurrent vasospasm of fingers and toes (may also affect ears, tip of nose) resulting in pallor, cyanosis, then erythema and a sense of fullness, in response to cold exposure or stress
• 80-90% are “primary Raynaud's disease”
• young female patients who have had Raynaud phenomenon alone for more than 2 years and have not developed any additional manifestations are at low risk for developing an autoimmune disease.¹⁾

• Raynaud's phenomenon is said to effect 3-5% of general population
• prevalence in Caucasians has been reported as being up to 11% of women and 8% of men

• risk factors:
  • genetic predisposition:
    • family history OR = 16.6
    • 55-64% hereditability ²⁾
    • associated with variations in gene NOS1 which as a known role in cold induced vascular responses ³⁾
  • migraine OR = 4.2
  • females OR = 1.65
  • smoking OR = 1.27
• usually occurs in young adults
• attacks triggered by exposure to cold and/or stress
• symmetric bilateral involvement
• no necrosis (very rare but may occur)
• may have increased risk of chilblains
• no detectable underlying cause
• normal lab findings
• negative anti-nuclear antibodies (ANA)

• frostbite
• vibration injury
• PVC exposure, or other organic solvent exposure
• lead, arsenic exposure

• scleroderma
  • 90% of patients have Raynaud's
• mixed connective-tissue disease:
  • 85% have Raynaud's
• systemic lupus erythematosus (SLE)
• dermatomyositis
• Sjögren's syndrome (SS)
• rheumatoid arthritis - 5% have Raynaud's
• vasculitis
• primary pulmonary hypertension

• hepatitis C virus
• hepatitis B virus
• mycoplasma

• lymphoma
• leukaemia
• multiple myeloma
• Waldenstrom's macroglobulinaemia
• polycythema
• cryoglobulinaemia
• lung adenoCa
• hyperviscosity syndromes

• acromegaly
• myxoedema
• diabetes mellitus
• phaechromocytoma
• Fabry disease
• paroxysmal nocturnal haemoglobinaemia

• combined oral contraceptive pill (OCP)
• ergot alkaloids
• bromocryptine
• beta adrenergic blockers
• antineoplastics
• cyclosporin
• alpha-interferon

• reflex sympathetic dystrophy
• carpal tunnel syndrome
• thoracic outlet syndrome
• peripheral vascular disease (PVD or PAD)
• thromboangiitis obliterans (Berger's)
• deep venous thrombosis (DVT)
• livedo reticularis
• acrocyanosis

• current pharmacological therapies for RP have limited efficacy and tolerability
• niacin / nicotinic acid / nicotinamide (vitamin B3) 500mg bd supplementation
  • may be of benefit and is generally well tolerated and inexpensive
  • appears to improve nailfold videocapillaroscopy (NVC) patterns and parameters in patients with primary Raynaud's (although small numbers of patients in the study)⁴⁾