Raynaud Phenomenon

Raynaud Phenomenon is a topic covered in the Washington Manual of Medical Therapeutics.

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General Principles

Raynaud phenomenon (RP) is a vasospasm of the digital arteries and can result in ischemia of the digits. It manifests as repeated episodes of color changes in the finger, toes, and less commonly ears and nose after cold exposure or emotional stress. The sequential color changes are white to blue to red, but this triad may not be seen in all patients. Well-demarcated pallor (white phase) is the most definitive phase. Patients may also experience numbness during the ischemic phase and pain during the rewarming phase.

Classification

  • RP can be classified as primary (idiopathic) or secondary if an underlying condition is present. Secondary RP is associated with multiple conditions and can be divided into different categories.
    • Systemic rheumatic disease: systemic sclerosis, MCTD, SLE, inflammatory myopathies, Sjogren syndrome, Burger disease, RA, vasculitis
    • Drugs or chemical induced: ergots, beta-blockers, bromocriptine, interferon-alfa, chemotherapeutic agents (vinblastine, bleomycin, cisplatin), vinyl chloride
    • Traumatic: pneumatic hammer operators, rock drillers, lumberjacks, etc.
    • Hyperviscosity syndrome: myeloproliferative disorders, paraproteinemia, leukemia, cryoglobulinemia, and cold agglutinins
    • Occlusive arterial disease: thrombotic/embolic arterial occlusion, thoracic outlet syndrome
    • Endocrinopathies: hypothyroidism, pheochromocytoma, carcinoid
    • Other: infections, complex regional pain syndrome, peripheral arteriovenous fistula
  • A thorough review of systems and physical examination is critical to differentiate between primary and secondary RP. Some of the other differences include:
    • Primary RP is more common in women and onset is typically between the ages of 15–30 years. These patients do not develop complications like ulceration, pitting, or gangrene. Nailfold capillary examination is normal and serologies are negative (although up to one-third may have low titer positive ANA).
    • Secondary RP may affect men or women. Onset tends to be in the third or fourth decade. Review of systems and physical examination may reveal signs or symptoms of an underlying condition. These patients are more prone to develop complications, nailfold capillary examination is abnormal, and serologies may be positive.

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General Principles

Raynaud phenomenon (RP) is a vasospasm of the digital arteries and can result in ischemia of the digits. It manifests as repeated episodes of color changes in the finger, toes, and less commonly ears and nose after cold exposure or emotional stress. The sequential color changes are white to blue to red, but this triad may not be seen in all patients. Well-demarcated pallor (white phase) is the most definitive phase. Patients may also experience numbness during the ischemic phase and pain during the rewarming phase.

Classification

  • RP can be classified as primary (idiopathic) or secondary if an underlying condition is present. Secondary RP is associated with multiple conditions and can be divided into different categories.
    • Systemic rheumatic disease: systemic sclerosis, MCTD, SLE, inflammatory myopathies, Sjogren syndrome, Burger disease, RA, vasculitis
    • Drugs or chemical induced: ergots, beta-blockers, bromocriptine, interferon-alfa, chemotherapeutic agents (vinblastine, bleomycin, cisplatin), vinyl chloride
    • Traumatic: pneumatic hammer operators, rock drillers, lumberjacks, etc.
    • Hyperviscosity syndrome: myeloproliferative disorders, paraproteinemia, leukemia, cryoglobulinemia, and cold agglutinins
    • Occlusive arterial disease: thrombotic/embolic arterial occlusion, thoracic outlet syndrome
    • Endocrinopathies: hypothyroidism, pheochromocytoma, carcinoid
    • Other: infections, complex regional pain syndrome, peripheral arteriovenous fistula
  • A thorough review of systems and physical examination is critical to differentiate between primary and secondary RP. Some of the other differences include:
    • Primary RP is more common in women and onset is typically between the ages of 15–30 years. These patients do not develop complications like ulceration, pitting, or gangrene. Nailfold capillary examination is normal and serologies are negative (although up to one-third may have low titer positive ANA).
    • Secondary RP may affect men or women. Onset tends to be in the third or fourth decade. Review of systems and physical examination may reveal signs or symptoms of an underlying condition. These patients are more prone to develop complications, nailfold capillary examination is abnormal, and serologies may be positive.

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