• S Zeni and F Ingegnoli.
• Dipartimento e Cattedra di Reumatologia dell'Università degli Studi, AO Istituto Ortopedico Gaetano Pini, Milano, Italy. zeni@gpini.it
• Reumatismo. 2004 Apr 1; 56 (2): 77-81.

AbstractRaynaud's phenomenon (RP) is a vasospastic disorder characterized by episodic color changes of blanching, cyanosis, and hyperemia in response to cold and/or emotional stress. Although most typically noted in the fingers, the circulation of the toes, ears, nose and tongue is also frequently affected. Population studies have shown that RP in adults is more common in women than men, with prevalence estimates ranging from 4% to 30%. Geographic variations in the prevalence reflect differences in climate. RP may be a primary or a secondary process. LeRoy and Medsger suggested criteria for primary RP: symmetric attacks, the absence of tissue necrosis, ulceration or gangrene, the absence of a secondary cause, negative antinuclear antibodies, normal nailfold capillaroscopy and a normal erythrocyte sedimentation rate. Secondary RP is characterized by an age of onset of more than 30 years, painful and asymmetric attacks, ischemic skin lesions, positive autoantibodies, capillaroscopic abnormalities and/or clinical features suggestive of connective tissue diseases (CTDs). Among the CTDs, systemic sclerosis has the highest frequency of RP. Finding a cause for RP requires a knowledge of the patient's occupational, smoking, drug history, physical examination, nailfold capillaroscopy, routine laboratory tests and autoantibodies. Furthermore, RP should be distinguished from acrocyanosis, a condition characterized by continuous cyanosis of the hands or feet that is aggravated by cold temperature. The most important instruction to the patient is abstinence from any smoking, offending drugs should be discontinued, and abrupt changes in temperature. If these measures are inadequate, calcium-channel blockers are the most widely used (nifedipine 30 mg up to 90 mg daily). Alternatively, sympatholytic agent (prazosin), angiotensin II -receptor type I antagonist (losartan), selective sertonin-reuptake inhibitor (fluoxetine) may be useful. In the severe cases the role of prostaglandins is well established, but standard therapeutic protocols are not jet available.

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