Specific anti-moesin antibodies could be detected in patients with pulmonary arterial hypertension associated with connective tissue diseases / 中华风湿病学杂志

ABSTRACT

Objective To detect the positive rate of anti-endothelial cell antibody (AECA) in patients with pulmonary arterial hypertension (PAH) associated with connective tissue diseases(CTD)and to investigate the specific target antigen.Methods Sera of 68 patients with CTD associated PAH were collected to detect AECA by Western blotting with extracted membrane protein of the endothelial cell line EA.hy926.Sera of 61 CTD patients without PAH,20 with idiopathic pulmonary arterial hypertension(IPAH),20 with chronic obstructive pulmonary diseases and pulmonary arterial hypertension (COPD-PAH) and 20 healthy donors were collected as controls.The correlation between PAH and specific bands of AECA was studied by X2 test.Liquid chromatography-electrospray ionization mass spectrography was used to detect the target antigens related to PAH associated with CTD.Results The specific molecular size of antigen was 78 000.The AECA-78 000positive rate of CTD patients with PAH was 79% (54/68).not significantly ditierent from that of CTD with glomerulonephritis(71%,15/21),but significantly higher than those of CTD with interstitial lung disease (ILD)(15%,3/20)and CTD without systemic involvement(P<0.01 and P<0.05 respectively).also higher than those of IPAH(8%,1/12).The AECA-78 000 was negative in COPD-PAH and healthy controls.The target antigen of AECA-78 000 was identified by proteomic techniques as moesin.Conclusion CTD patients with different target organ involvement have different AECA-78 000 positive rates,which could be frequently detected in CTD associated PAH and those with glomerulonephritis.The common antigen is moesin.