Autoimmunity and inflammation have been revealed in the pathogenesis of connective tissue disease associated pulmonary arterial hypertension (CTD-PAH). It is suggested by literatures that there may be several types of serum biomarkers exclusively for CTD-PAH rather than those for idiopathic PAH. These promising markers include: (1) autoantibodies, such as anti-endothelial antibody, anti-fibroblast antibody,anti-beta2-glycoprotein 1 (GP1) antibody, anti-U1 and U3 RNP antibody; (2) inflammatory markers including chemokines (e.g. CX3CL1, CCL2 and RANTEs), growth factors (e.g. platelet-derived growth factor), and (3) others biomarkers, such as N-terminal brain natriuretic peptide (NT-BNP), endoglin, etc. Further investigation and research are in demand to confirm their efficacy in predicting PAH secondary to CTD.%结缔组织病相关肺动脉高压(CTD-PAH)因其发病机制存在自身免疫和炎症反应,故其病情评估指标除适用于特发性肺动脉高压的标记物外,文献提示还可能存在以下几种血清学标记物:(1)自身抗体,如抗内皮细 胞抗体、抗成纤维细胞抗体、抗β2-糖蛋白1(β2-GP1)抗体以及抗U1RNP和U3RNP抗体;(2)炎症因子,包括趋化因子(如CX3CL1,CCL2和RANTEs)、生长因子(如血小板源性生长因子等);(3)其他血清标记物如N-末端尿钠肽原、endoglin等.这些标记物可能成为提示或预测CTD-PAH更为特异的标记物,值得进一步探讨和证实.
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