Anti-endothelin receptor type A autoantibody in lupus associated pulmonary arterial hypertension
10.3760/cma.j.issn.1007-7480.2015.03.004
- VernacularTitle:抗内皮素A型受体抗体在狼疮相关肺动脉高压中的致病性
- Author:
Jiangfeng ZHAO
;
Li GUO
;
Yi CHEN
;
Shuming PAN
;
Shuang YE
- Publication Type:Journal Article
- Keywords:
Receptor,endothelin A;
Autoantibodies;
Lupus erythematosus,systemic;
Hypertension,pulmonary
- From:
Chinese Journal of Rheumatology
2015;19(3):156-159,后插1
- CountryChina
- Language:Chinese
-
Abstract:
Objective To investigate autoantibody against endothelin receptor type A (ENRA-Ab) in patients with systemic lupus erythematosus associated pulmonary arterial hypertension (SLE-PAH).The possibility of autoantibody-mediated pathogenesis in the development of SLE-PAH has also been explored.Methods ENRA-Ab in the serum of SLE-PAH and controls were detected by using a human ETRA epitope peptide-based ELISA.The clinical relevance of ENRA-Ab in SLE-PAH was analyzed.Proliferation of vascular smooth muscle cells (SMCs) and permeability of endothelial cells in vitro under the stimulation of polyclonal ENRA-Ab IgG were assessed.The expressions of PAH-related markers, i.e., 5-HTT, PDGFR-b, VEGF-A and PDGF-B were measured by qPCR.The effect of ENRA-Ab in vivo was also determined in a suboptimaldose monocrotaline-induced model with the assessment of right ventricle hypertrophy index and pathology parameters.Independent t-test, Tukey-Kramer test of variance analysis and Pearson' s correlation analysis were used for statistical analysis.Results ENRA-Abs was presented in a higher occurrence in SLE-PAH (35/85,41％) compared with controls (0/60;0, 13/80, 16％).There was a significant correlation between ENRA-Ab and echocardiograph estimated pulmonary arterial systolic pressure (r=0.392, P=0.002) in SLE-PAH.ENRA-Ab could promote SMCs proliferation, disrupt endothelial barrier and up-regulate PAH-related markers expression,which could be blocked in the presence of ETR antagonist.ENRA-Ab aggravated right ventricle hypertrophy and vascular remodeling in vivo.Conclusion ENRA-Ab is a new biomarker, in SLE-PAH, which may mediate PAH development in SLE.
