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KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • One or more digits blanch with cold exposure or emotional upset and subsequently show hyperemia when warmed

  • Nail fold capillaries are normal by microscopy in primary Raynaud’s phenomenon

  • Focal digital-tip necrosis

GENERAL CONSIDERATIONS

  • Primary Raynaud’s phenomenon:

    • – Is 10 times more common in women than in men

    • – Starts around puberty

  • Raynaud-type symptoms occur in about 20% of women in northern countries

  • Secondary Raynaud’s phenomenon:

    • – Starts at an older age

    • – Is due to vasculitis or coagulopathies

    • – Is more likely to result in ischemic digital injury

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • Exposure to cold, emotional upset, or smoking causes 1 or more digits to blanch with subsequent hyperemia on rewarming

  • Primary Raynaud’s phenomenon usually spares the thumb

PHYSICAL EXAM FINDINGS

  • Digital-tip necrosis can be seen in secondary Raynaud’s phenomenon

  • Physical findings of an autoimmune disease such as arthritis may be found

DIFFERENTIAL DIAGNOSIS

  • Primary disease versus secondary causes, such as collagen vascular diseases, vasoactive drugs, and blood viscosity disorders

DIAGNOSTIC EVALUATION

LABORATORY TESTS

  • Patients with primary disease have completely normal laboratory tests

  • In secondary disease, the CBC, antinuclear antibody titer, rheumatoid factor, erythrocyte sedimentation rate, von Willebrand factor antigen, cryoglobulin, cryofibrnogen, and cold agglutinin titers may be abnormal

IMAGING STUDIES

  • Capillary microscopy: useful for distinguishing primary from secondary Raynaud’s phenomenon:

    • – The capillaries are normal in the primary disease

    • – Various degrees of capillary loss are seen in the secondary form

TREATMENT

MEDICATIONS

  • Use vasoactive drugs, such as calcium channel blockers, eg, amlodipine 2.5–5 mg PO daily

SURGERY

  • Digital sympathectomy in extreme cases

ONGOING MANAGEMENT

COMPLICATIONS

  • Digital necrosis

PROGNOSIS

  • Excellent in primary disease

PREVENTION

  • Stop smoking

  • Avoid cold exposure (eg, wear gloves)

  • Avoid rings on affected digits

RESOURCES

REFERENCE

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Wigley  FM, Flavahan  NA. Raynaud's phenomenon. N Engl J Med. 2016;375(6):556–565.  [PubMed: 27509103]

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