Raynaud phenomenon

Last updated: August 13, 2022

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Raynaud phenomenon (RP) is an exaggerated vasoconstrictive response of distal arteries and arterioles (most commonly in the fingers and toes) to cold or emotional stress. Primary RP is idiopathic, whereas secondary RP is caused by underlying systemic diseases (e.g., mixed connective tissue disease, vasculitides, medications). Both types typically manifest with the sequential discoloration of fingers and/or toes from white (ischemia) to purple-blue (hypoxia) to red (reactive hyperemia). Episodes of vasoconstriction usually last for 15–20 minutes after removal of the trigger. Ischemic episodes in secondary RP can be more prolonged, leading to tissue loss (ischemic ulcers) and rarely, gangrene. Trigger avoidance is the main aspect of managing RP. In patients with secondary RP, the underlying etiology should be identified and treated. Pharmacotherapy (preferably with calcium channel blockers) may be considered for RP refractory to trigger avoidance. Intravenous prostaglandins or interventional therapy (e.g., selective digital sympathectomy) may be required for patients with severe or refractory disease.

Overview [1][2][3]

  • Vasospastic attack triggered by cold or emotional stress
  • More common in female individuals

Primary Raynaud phenomenon (previously called Raynaud disease)

  • Idiopathic (no identifiable vascular changes); possible genetic susceptibility [1]
  • Vasospasms of the digital arteries and arterioles
  • Onset usually < 30 years of age

Secondary Raynaud phenomenon (previously called Raynaud syndrome)

Classic presentation [1][4][5]

An episode does not always involve all three phases; often, blood flow is restored without causing reactive hyperemia. [1]

Duration [1][5]

  • Vasospastic episodes are usually reversible.
  • Symptoms normally subside within an hour (typically 15–20 minutes after cessation of trigger exposure). [1]
  • Can potentially last for several hours

Associated findings [1][5]

Trophic changes are uncommon in primary RP. Irreversible ischemia with tissue damage indicates secondary RP and requires further investigation to identify the underlying cause. [1]

General principles [1][4][6][7]

Diagnostic studies [1][6][8]

Findings are described in the table below.

Differentiation between primary and secondary RP

Primary vs. secondary Raynaud phenomenon [4][6]
Primary RP Secondary RP

Characteristic features

  • Onset < 30 years of age
  • Episodes entirely reversible
  • Thumb is typically spared
  • No tissue loss
  • No signs of CTD

Laboratory studies

Nailfold capillaroscopy

  • Normal pattern
    • Uniform and regularly distributed
    • Hairpin capillary morphology
  • Abnormal (scleroderma pattern)
    • Decreased capillary density (i.e., dropout)
    • Dilated “giant” capillaries (diameter ≥ 50 μm)
    • Abnormal architecture
    • Hemorrhages

It is critical to evaluate for secondary RP because missing early CTD or other associated causes can have serious consequences.

Perniosis (chillblains) [11][12][13]

  • Erythrocyanotic papules or nodules, predominantly on the hands, fingers, toes, legs, and face on exposure to cold
  • Lesions appear 12–24 hours after exposure to cold and last for 1–3 weeks.
  • Typically seasonal (colder months)
  • See “Nonfreezing cold injuries” for details.

Acrocyanosis [14][15]

  • No triphasic color response like in RP, nonparoxysmal manifestation (often persistent)
  • Recurrent or frequently persistent bluish/cyanotic discoloration of the peripheral extremities
  • Most commonly affects the hands; more rarely the feet, ears, nose, lips, and nipples
  • Caused by decreased oxyhemoglobin due to vasospasm
  • Usually triggered and/or aggravated by exposure to cold temperatures, most cases improve in summer.
  • Trophic changes and ulceration are extremely rare.
  • May be primary (idiopathic) or secondary to neurologic, autoimmune, infective, metabolic, or other causes

Erythromelalgia [16][17]

  • Rare condition characterized by episodes of hyperperfusion that affect the feet and less commonly the hands
  • Symptoms include intense burning pain, erythema, warmth, and swelling of the affected areas.
  • Triggers include warmth and physical activity.
  • May be primary (genetically determined) or secondary to myeloproliferative disorders, CTD, cancer, infections, or poisoning.

Peripheral artery disease (PAD)

  • Exercise-induced pain (may also be persistent)
  • Cold and pale extremities
  • Diminished or absent radial or ulnar artery pulse
  • Can be a cause of secondary RP (functional vasospasm), but must be considered as an independent differential diagnosis.

Acute arterial occlusion of an extremity

Thoracic outlet syndrome [9][18]

The differential diagnoses listed here are not exhaustive.

Approach [1][4]

  • First line management in all patients: trigger avoidance and lifestyle measures to minimize vasospastic attacks
  • Persistent symptoms
    • Consider pharmacotherapy.
    • Consider interventional therapies in patients with refractory disease and surgical treatment when indicated.
  • Secondary RP
    • Identify and treat the underlying cause.
    • Specialist referral (e.g., immunology, rheumatology) for further management.

Trigger avoidance [1][4]

  • Avoidance of cold (e.g., wearing warm gloves or multiple layers of clothing)
  • Stress management (e.g., anxiety attacks, episodes of irritability/anger)
  • Minimize vibration exposure (e.g., occupational use of power hand tools such as jackhammers)
  • Smoking cessation (smoking promotes vasoconstriction)
  • Discontinuation of medications that may trigger attacks (e.g., β-blockers, ergotamine, oral contraceptives)

Cold avoidance and stress management are key aspects of managing RP. [4][7]

Pharmacotherapy [1][4][6][7]

There is a paucity of evidence on the efficacy of pharmacotherapy in RP and currently, no drugs have received FDA approval for treatment of the condition. [1][7][19][20]

Pharmacotherapy for RP often causes significant side effects (e.g., hypotension). Start all medications at a low dose and gradually increase as tolerated. [1]

Interventional therapies and surgery [1][4][6]

  • Indications: refractory or severe RP (e.g., critical digital ischemia, as evidenced by persistent ulceration or gangrene)
  • Procedures
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  10. Fayyaz A, Igoe A, Kurien BT, et al. Haematological manifestations of lupus. Lupus Sci Med. 2015; 2 (1): p.e000078-e000078. doi: 10.1136/lupus-2014-000078 . | Open in Read by QxMD
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  14. Das S, Maiti A. Acrocyanosis: An overview. Indian J Dermatol. 2013; 58 (6): p.417-420. doi: 10.4103/0019-5154.119946 . | Open in Read by QxMD
  15. Wollina U, Koch A, Langner D, et al. Acrocyanosis - A Symptom with Many Facettes. Open Access Maced J Med Sci. 2018; 6 (1): p.208-212. doi: 10.3889/oamjms.2018.035 . | Open in Read by QxMD
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  21. Chung L, Shapiro L, Fiorentino D, et al. MQX-503, a novel formulation of nitroglycerin, improves the severity of Raynaud's phenomenon: a randomized, controlled trial. Arthritis Rheum. 2009; 60 (3): p.870-7. doi: 10.1002/art.24351 . | Open in Read by QxMD
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