Raynaud's phenomenon in common rheumatic diseases

Raynaud's phenomenon (RP) is caused by a reversible spasm of the smallest arteries and the arterioles of the fingers and toes. Two forms of RP have been described: primary and secondary. Secondary RP is often present in patients with rheumatic diseases. This review presents the characteristics of the clinical pattern, the immunological profile and the capillaroscopic pattern in patients with primary and secondary RP in common rheumatic diseases. Attention is paid to standard examinations usually used in RP patients. Primary RP appears to be more common in women beginning usually at puberty. This disorder is caused by vasospasm; no abnormalities of the endothelium are observed. Primary RP shows benign progression. Laboratory tests - erythrocyte sedimentation rate (ESR) and antinuclear antibody test (ANA) - are normal. Capillaroscopy is normal too. Secondary RP tends to begin later in life. Of the rheumatic diseases, scleroderma is the one that is the most often associated with RP (in 90-95% of all cases). The pathogenesis of secondary RP in scleroderma is explained with abnormalities of the endothelium through different mechanisms. Motor ulcerations are frequently observed. Anticentromeric and antitopoizomeric antibodies get positive. Capillaroscopy appears to be very important for the early diagnosis and prognosis of scleroderma. The capillaroscopic pattern is abnormal - enlarged capillaries, hemorrhages and avascular areas are observed. Part of the patients with systemic lupus erythematosus, Sjogren's syndrome and polymyositis / dermatomyositis develops secondary RP and it usually shows benign progression. RP is the main symptom in mixed connective tissue disease and trophic abnormalities of the fingers are frequently observed. Elevated anti-U1-RNP antibody titers and an abnormal capillaroscopic pattern are specific for the condition. In older patients isolated RP may represent a paraneoplastic manifestation. RP is frequently found in the rheumatologic practice. The differentiation of the primary form from the secondary one is essential because of the differences concerning the heaviness of the disorder, the prognosis and the therapeutic approach.