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AT-A-GLANCE

AT-A-GLANCE

  • Raynaud phenomenon is a vascular disorder characterized by recurrent episodic attacks of digital ischemia provoked by exposure to cold or emotional stress.

  • Affects up to 10% of the population, 4:1 female-to-male ratio.

  • Classified as primary (idiopathic) and secondary (underlying disease or cause present) forms; severity ranges from mild/benign to severe with loss of tissue and risk of amputation.

  • Connective tissue diseases, particularly systemic sclerosis, are among the most common underlying causes of secondary Raynaud phenomenon.

  • Behavioral modification, pharmacologic therapies, and surgical interventions are effective at reducing the frequency and severity of attacks.

Raynaud phenomenon (RP) is a common disorder characterized by recurrent attacks of arterial vasoconstriction resulting in hypoperfusion of the digits and acral tissues. Episodes of RP are triggered by exposure to cold or, less commonly, emotional stress. Classically, RP exhibits a triphasic sequence of color changes of the digits of the hands and/or feet: well-demarcated pallor (corresponding to vasoconstriction and ischemia), followed by cyanosis (venous stasis), and, finally, erythema (compensatory reperfusion) upon rewarming.1 However, not all patients demonstrate this classic triad of color changes.

Depending on the presence or absence of an underlying disorder, 2 forms of RP are recognized and usually display divergent disease courses. Primary (idiopathic) RP represents an exaggerated physiologic response to cold or emotional stimuli caused by functional changes in blood vessels and their innervation.2 By definition, primary RP does not result in tissue injury. Secondary RP, which occurs as a result of a systemic disorder or drug exposure, is distinguished by a more aggressive course that can lead to profound tissue ischemia and eventuate in cutaneous ulceration, scarring, or digital gangrene. Management of RP centers on behavioral changes to avoid attacks and on pharmacologic and nonpharmacologic therapies to allay symptoms and reduce morbidity. When present, treatment of the underlying cause remains a critical component of managing secondary disease.

EPIDEMIOLOGY

Precise epidemiologic data on RP are lacking. True estimates are biased by underreporting because many patients with RP never seek medical attention and by the greater attention that has been given to secondary forms of the disease. RP likely affects up to 10% of the general population and 90% of patients with systemic sclerosis (SSc).3-5 Despite study biases, cases of primary RP far outnumber those of secondary disease and comprise up to 90% of all cases.6,7 Symptoms of RP often develop during the second decade of life, and there is a strong female predominance, with an estimated 4:1 female-to-male prevalence ratio. Increases in the frequency and severity of attacks during menstrual cycles suggest that female hormones play a role in the pathogenesis of RP.8 Differences in seasonal skin temperatures, alcohol use, age, smoking status, and marital status between women and men also explain the sex differences observed.9,10 Familial aggregation has been noted in some series, substantiating a role for genetic predisposition.11...

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