Antibodies in scleroderma: direct pathogenicity and phenotypic associations.
Antibodies in scleroderma: direct pathogenicity and phenotypic associations.
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DOI：
10.1007/s11926-004-0061-9
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发表时间：
2004-04-01
影响因子：
5
通讯作者：
Utz, Paul J
中科院分区：
文献类型：
--
作者：
Chung, Lorinda;Utz, Paul J
硬皮病是一种自身免疫性疾病，涉及内皮细胞损伤和成纤维细胞过度产生细胞外基质。硬皮病患者血清中存在的几种自身抗体，包括抗内皮细胞抗体、抗成纤维细胞抗体、抗基质金属蛋白酶抗体和抗纤维蛋白-1抗体，可能直接导致疾病的发病机制。硬皮病的特征还在于存在与特定表型相关的抗核抗体和抗核仁抗体。这些包括抗拓扑异构酶-I、抗着丝粒、抗组蛋白、抗多发性肌炎/硬皮病、抗Th/To、抗U3-小核仁核糖核蛋白颗粒、抗U1-小核核糖核蛋白颗粒、抗RNA聚合酶和抗B23抗体。与其他自身免疫性疾病典型相关的其他抗体，如抗磷脂抗体、抗神经细胞质抗体和抗线粒体抗体，也在硬皮病患者中有描述。本文将总结与硬皮病相关的各种自身抗体，其假定的致病作用，及其表型相关性。
Scleroderma is an autoimmune disease involving endothelial cell damage and fibroblast overproduction of extracellular matrix. Several autoantibodies present in the sera of patients with scleroderma, including anti-endothelial cell, antifibroblast, anti-matrix metalloproteinase, and antifibrillin-1 antibodies, may directly contribute to disease pathogenesis. Scleroderma also is characterized by the presence of antinuclear and antinucleolar antibodies, which correlate with particular phenotypes. These include antitopoisomerase-I, anticentromere, antihistone, anti-polymyositis/scleroderma, anti-Th/To, anti-U3-small nucleolar ribonucleoprotein particle, anti-U1-small nuclear ribonucleoprotein particle, anti-RNA polymerase, and anti-B23 antibodies. Other antibodies classically associated with other autoimmune diseases, such as antiphospholipid, antineutrophil cytoplasmic, and antimitochondrial antibodies, also have been described in patients with scleroderma. This review will summarize the various autoantibodies associated with scleroderma, their putative pathogenic roles, and their phenotypic correlations.