Lambova Sevdalina N, Kuzmanova Stefka I
Clinic of Rheumatology, Medical University, Plovdiv, Bulgaria.
Folia Med (Plovdiv). 2006;48(3-4):22-8.
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.
雷诺现象（RP）是由手指和脚趾的最小动脉及小动脉的可逆性痉挛引起的。RP有两种类型：原发性和继发性。继发性RP常见于风湿性疾病患者。本综述介绍了常见风湿性疾病中原发性和继发性RP患者的临床模式特征、免疫学特征和毛细血管镜检查特征。重点关注RP患者通常进行的标准检查。原发性RP在女性中似乎更常见，通常始于青春期。这种疾病是由血管痉挛引起的；未观察到内皮异常。原发性RP呈良性进展。实验室检查——红细胞沉降率（ESR）和抗核抗体试验（ANA）——均正常。毛细血管镜检查也正常。继发性RP往往在生命后期开始。在风湿性疾病中，硬皮病是最常与RP相关的疾病（在所有病例中占90 - 95%）。硬皮病继发性RP的发病机制通过不同机制以内皮异常来解释。经常观察到活动性溃疡。抗着丝点抗体和抗拓扑异构酶抗体呈阳性。毛细血管镜检查对于硬皮病的早期诊断和预后似乎非常重要。毛细血管镜检查模式异常——观察到毛细血管扩张、出血和无血管区。部分系统性红斑狼疮、干燥综合征和多发性肌炎/皮肌炎患者会出现继发性RP，通常呈良性进展。RP是混合性结缔组织病的主要症状，经常观察到手指的营养异常。抗U1 - RNP抗体滴度升高和异常的毛细血管镜检查模式是该疾病的特异性表现。在老年患者中，孤立性RP可能是一种副肿瘤表现。RP在风湿病临床实践中很常见。区分原发性和继发性形式至关重要，因为这两种形式在疾病严重程度、预后和治疗方法上存在差异。
