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ESSENTIALS OF DIAGNOSIS

  • Raynaud phenomenon (RP) is an exaggeration of the normal vasospastic reaction to cold environments or emotional stress*.

  • Characterized by well-demarcated color changes (pallor, cyanosis, hyperemia) involving the peripheral digits (fingers and toes).

  • Classified clinically into primary or secondary forms.

  • Primary RP is idiopathic and functional in nature, the integrity of the blood vessel architecture is preserved.

  • Secondary RP is associated with an underlying structural vasculopathy and may be complicated by digital ischemia, recurrent digital ulcerations, rapid deep tissue necrosis, and amputation.

  • The principal approach in the management of RP entails preventative measures through the avoidance of cold environments and use of protective clothing to maintain adequately warm core and peripheral body temperatures.

  • Therapeutic agents are indicated if quality of life is affected adversely or if there are complications of tissue ischemia, including digital ulcers.

General Considerations

Raynaud phenomenon (RP) is classified into two categories: primary and secondary. Primary RP accounts for the majority (80–90%) of cases. It occurs in the absence of an identifiable disease and is due to vasospastic events precipitated by exposure to cold environments or emotional stress. Primary RP occurs more commonly in otherwise healthy females, with an onset of symptoms around 15–30 years of age. A positive family history of RP in first-degree relatives is reported in 30–50% of patients. The RP attacks are typically bilateral and symmetrical, affecting the hands. The thumb is generally spared. It is not complicated by progression to tissue necrosis or gangrene. Nailfold capillaroscopy (see later) and physical examination findings are normal. If a patient meets criteria for primary RP and no new symptoms develop over 2 years of follow-up, the development of secondary disease is unlikely to occur. The presence of abnormal nailfold capillaries on capillaroscopy or specific autoantibodies are strong predictors of secondary RP associated with an underlying autoimmune disease (Overbury et al, 2015).

Secondary RP is seen in a variety of disorders that disturb normal vascular reactivity. It is often observed in patients with autoimmune diseases, particularly systemic sclerosis (SSc), systemic lupus erythematosus (SLE), Sjögren syndrome, and dermatomyositis. In 10–20% of cases, it presents as the initial manifestation of an underlying autoimmune disease but often it goes unrecognized for years and is not appreciated fully until other disease manifestations occur (Goundry et al, 2012). RP is associated with pathology that alters regional blood flow by various mechanisms, including damaging blood vessels, interfering with neural circulatory control, and changes in the physical properties of blood or the levels of circulating mediators that regulate digital and cutaneous circulation (Herrick, 2012). Patients with secondary RP generally have more severe, painful RP that may be associated with digital ulceration and gangrene. In fact, the occurrence of such tissue injury places a patient’s case of RP clearly in the secondary RP category.

Pathogenesis

Involvement of the skin of the fingers, toes, ...

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