Objective To investigate the correlation between autoantibodies against endothelin 1 receptor type A(ETRA)and connective tissue disorder(CTD)-associated pulmonary arterial hy-pertension(PAH).Methods Plasma levels of antinuclear antibodies(ANA),anti-extractable nu-clear antigen antibodies(anit-nRNP,anit-Sm,anti-SCl-70,anti-ds-DNA,anti-CENP),N-terminal pro-B-type natriuretic peptide(NT-proBNP),and anti-endothelin antibodies were measured in 60 CTD patients with PAH(CTD-PAH group)and 60 CTD patients without PAH(CTD group).In addition,forced expiratory volume in 1 second(FEV1),forced vital capacity(FVC),carbon mon-oxide diffusing capacity(DLco),six minute walking test(6 MWT)and pulmonary arterial systolic pressure(PASP)were determined in both groups.To explore the possibility of anti ETRA anti-body as an assistant biomarker for early diagnosis of CTD-PAH.Results In CTD-PAH group, ETRA autoantibodies(+)appeared in 29 cases(48.3%)and ETRA autoantibodies(-)in 31 ca-ses(51.7%).In CTD group,ETRA autoantibodies(+)appeared in 4 cases(6.7%)and ETRA autoantibodies(-)in 56 cases(93.3%).Compared with CTD group,the proportions of ETRA autoantibodies(+)and anti-nRNP(+)and the levels of PASP and NT-proBNP significantly in-creased but the 6 MWDS and levels of FVC and DLco obviously decreased in CTD-PAH group (P <0.05).ETRA autoantibody titers were positively correlated with PASP(r = 0.2246,P =0.0281)and NT-proBNP levels(r=0.3306,P =0.0346),but negatively correlated with 6 MWT (r=-0.3274,P =0.0445)in CTD-PAH group.Conclusion ETRA autoantibodies can be used as new biomarkers to assess the severity of PAH and cardiopulmonary dysfunction in patients with CTD-PAH.%目的 观察抗内皮素A型受体(ETRA)抗体与弥漫性结缔组织病(CTP)相关肺动脉高压(CTD-PAH)发病及疾病严重程度之间的关系,从而探讨抗ETRA抗体作为协助早期诊断CTD-PAH生物标记物的可能性.方法 选择CTD-PAH患者(CTD-PAH组)60例和CTD患者(CTD组)60例,分别检测2组血浆抗核抗体(ANA)、抗可溶性抗原(ENA)抗体谱(抗nRNP、抗Sm、抗SCl-70、抗ds-DNA、抗CENP)、N末端B型利钠肽原(NT-proBNP)和抗ETAR抗体的水平,肺功能[第1秒用力呼气量(FEV1)、用力肺活量(FVC)、一氧化碳弥散量(DLco)]、6 min步行试验(6 MWT)及肺动脉收缩压(PASP)的水平.结果 CTD-PAH组的抗ETRA抗体(+)29例(48.3%)、抗ETRA抗体(-)31例(51.7%).CTD组的抗ETRA抗体(+)4例(6.7%)、抗ETRA抗体(-)56例(93.3%).CTD-PAH组中抗ETRA抗体(+)和抗nRNP(+)的比例以及PASP、血浆NT-proBNP水平均明显高于CTD组,而6 MWT较CTD组短,FVC、DLco水平均明显低于CTD组(均P<0.05).CTD-PAH组血浆抗ETRA抗体水平与PASP呈正相关(r=0.2246,P<0.05),CTD-PAH组抗ETRA抗体滴度与6 MWD呈负相关(r=-0.3274,P<0.05),CTD-PAH组抗ETRA抗体滴度与血浆NT-proBNP水平呈正相关(r=0.3306,P<0.05).结论 抗ETRA抗体可用于CTD-PAH患者肺动脉压、心肺功能损害严重程度的评估,抗ETRA抗体可作为新的生物标记物用于CTD-PAH的诊断.
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