R aynaud phenomenon is a common circulatory disorder, which predominantly results in paroxysmal, temporary ischemia of the fingers or toes. Taking geographic and climatic variations into account, the prevalence of Raynaud phenomenon ranges between 3% and 10%.
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Clinical Perspective on p 517Raynaud phenomenon can be subclassified as primary Raynaud phenomenon, when no underlying disorder is present, or as secondary Raynaud phenomenon, which occurs as a result of another local or systemic disease. 4 Because Raynaud phenomenon might precede a systemic autoimmune disease of connective tissues, the diagnostic workup primarily focuses on the detection of early signs of an emerging connective tissue disease. 5,6 Therefore, nailfold capillaroscopy and laboratory tests comprising immunologic markers, such as antinuclear antibodies (ANA) and respective subsets, are routinely obtained in patients with incipient Raynaud phenomenon. 4,[6][7][8][9][10][11] Interestingly, recent data suggested an age-dependent association between Raynaud phenomenon and mortality, which might be attributable to an underlying vascular abnormality in Raynaud phenomenon.12 Although nailfold capillaroscopy and autoantibodies potentially indicate the emergence of an underlying systemic disorder, data on the impact of nailfold capillary aberrations and autoantibodies on mortality are limited to small groups of selected patients. 13 Whether and to what extent particular nailfold capillary aberrations and autoantibody expression are associated with all-cause mortality in patients with Raynaud phenomenon and without previously known connective tissue disease is not known.Background-In incipient Raynaud phenomenon, nailfold capillaroscopy and autoantibody tests are obtained to screen for an emerging connective tissue disease. Whether the presence of abnormal nailfold capillaries and autoantibodies are related to mortality in patients with incipient Raynaud phenomenon is not known. Methods and Results-In 2958 consecutive patients (78% women, median age 45 years) with incipient Raynaud phenomenon without previously known connective tissue disease, nailfold capillaroscopy and laboratory tests for antinuclear antibodies (ANA) and ANA subsets were obtained at initial presentation. During a median follow-up period of 9.3 years, 227 women (9.9% of female patients) and 129 men (20% of male patients) with Raynaud phenomenon died. In comparison with a demographically matched standard population, survival was poorer in patients with Raynaud phenomenon (log-rank test P<0.0001). In patients with Raynaud phenomenon, mortality was higher in men than in women (P<0.0001, Cox proportional hazards model). In women, the presence of abnormal nailfold capillaries, ANA, and anti-Scl-70 antibodies were related to an increase in all-cause mortality. The conjoint presence of abnormal nailfold capillaries and autoantibodies was associated with the highest mortality rates. In men, abnormal nailfold capillaries, and ANA and ANA subsets, as well, were not related to survival. In bo...