THE CREST SYNDROME - A DISTINCT SEROLOGIC ENTITY WITH ANTICENTROMERE ANTIBODIES
THE CREST SYNDROME - A DISTINCT SEROLOGIC ENTITY WITH ANTICENTROMERE ANTIBODIES
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DOI：
10.1016/0002-9343(80)90462-3
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发表时间：
1980-01-01
影响因子：
5.9
通讯作者：
KINSELLA, TD
中科院分区：
文献类型：
--
作者：
FRITZLER, MJ;KINSELLA, TD
CREST综合征是系统性硬化症的一种变体，其特征是存在钙质沉着、雷诺现象、食管运动异常、指端硬化和毛细血管扩张。检查了 27 名 CREST 综合征患者的血清中是否存在抗核抗体。在 27 份血清中，26 份 (98%) 含有高滴度 (> 1:80) 的抗体，该抗体可在人喉癌细胞系 (HEp-2) 上产生离散的免疫荧光斑点图案。该抗体与中期染色体的着丝粒区域发生反应。在 14 名雷诺氏病患者中的 3 名、60 名系统性红斑狼疮患者中的 1 名、26 名患有弥漫性硬皮病的系统性硬化症患者中的 3 名以及 15 名混合结缔组织病患者中的 1 名中发现了这种抗体。在类风湿性关节炎、干燥综合症或线性硬皮病患者的血清中未检测到该抗体。与 CREST 综合征组年龄和性别匹配的骨关节炎患者没有抗着丝粒抗体。 CREST 综合征患者的血清中未发现其他结缔组织疾病中发现的自身抗体（抗 DNA、抗 RNP [核糖核蛋白] 和干燥综合征抗原 B（抗 SS-B））。一份病例报告说明了当雷诺现象早于 CREST 综合征的临床诊断时抗着丝粒抗体的出现。
The CREST syndrome is a variant of systemic sclerosis, characterized by the presence of calcinosis, Raynaud''s phenomenon, esophageal motility abnormalities, sclerodactyly and telangiectasia. The serums of 27 patients with the CREST syndrome were examined for the presence of antinuclear antibodies. Of 27 serums, 26 (98%) contained high titers (> 1:80) of an antibody that produces a discrete speckled pattern of immunofluorescence on a human laryngeal carcinoma cell line (HEp-2). The antibody reacted with the centromeric region of metaphase chromosomes. This antibody was found in 3 of 14 patients with Raynaud''s disease, in 1 of 60 patients with systemic lupus erythematosus, in 3 of 26 patients with systemic sclerosis with diffuse scleroderma and in 1 of 15 patients with mixed connective tissue disease. The antibody was not detected in the serums of patients with rheumatoid arthritis, Sjogren''s sicca complex or linear scleroderma. Patients with osteoarthritis who were age- and sex-matched to the group with the CREST syndrome did not have anti-centromere antibodies. Autoantibodies found in other connective tissue diseases (anti-DNA, anti-RNP [ribonucleoprotein] and Sjogren''s syndrome antigen B (anti-SS-B)) were not found in serums from patients with the CREST syndrome. A case report illustrating the appearance of the anticentromere antibody at a time when Raynaud''s phenomenon antedated the clinical diagnosis of CREST syndrome is presented.