N Engl J Med 2016; 375:556-565August 11, 2016DOI: 10.1056/NEJMra1507638
In his 1862 thesis, Maurice Raynaud describes the condition afflicting a 26-year-old female patient: “Under the influence of a very moderate cold . . . she sees her fingers become ex-sanguine, completely insensible, and of a whitish yellow color. This phenomenon happens often without reason, lasts a variable time, and terminates by a period of very painful reaction, during which the circulation is re-established little by little and recurs to the normal state.”1 The term “Raynaud’s disease” was used to describe these vascular events until Hutchinson, who argued that multiple etiologic factors could be responsible, introduced the concept of “Raynaud’s phenomenon.”2Although results vary according to sex, local environmental climate,3 and work exposures,4 most population-based surveys estimate the prevalence of the disorder in the general population at 3 to 5%.5 We currently classify patients into two groups: those with primary Raynaud’s phenomenon, which is diagnosed when no underlying disease is found; and those with secondary Raynaud’s phenomenon, which is diagnosed when there is associated disease. This review provides an update on new insights into the mechanism and pathogenesis of Raynaud’s phenomenon and on current approaches to the management of this disorder.6
DIAGNOSIS AND CLINICAL FEATURES
Although laboratory testing provides important information about the hemodynamic and physiological features of Raynaud’s phenomenon, clinical assessment by means of history or direct observation remains the best approach for diagnosis.
Keep reading at the NEJM