Potential Biomarkers For Detecting Pulmonary Arterial Hypertension

[Objective]The association between autoimmunity and pulmonary artery hypertension(PAH) has been appreciated for>40years, but biomarkers for the diagnosis of this disease is not yet known. Here we examined the serum levels of RDW, anti-moesin antibody, and endothelin-1in patients with systemic lupus erythematosus (SLE) or systemic sclerosis (SSc), two popular research models of connective tissue disease(CTD) associated PAH (CTD-PAH). We also studied the expression of moesin on peripheral blood mononuclear cells (PBMC) and its relationship with anti-moesin antibodies.[Methods]1) Hemodynamic variables, along with demographic, clinical, laboratory, and red cell distribution width (RDW) were recorded from77SSc patients and89SLE patients in Peking Union Medical College Hospital.79health controls’ blood samples were collected from the Laboratory department.2) Enzyme-linked immunosorbent assay was used to determine the serum levels of anti-moesin antibody and endothelin-1.3) Flow cytometry was used to detect changes in moesin expression on the surface of PBMC, as well as the total amount of moesin in PBMC.[Results]1) RDW level were raised in patients with PAH (P=0.013). It correlated significantly with WHO functional class (P=0.002), six minutes walk distance (P=0.025) and NT-BNP(P=0.038).2) Anti-moesin antibody was significantly raised in CTD-PAH (P=0.000). It detected PAH with47%sensitivity and87%specificity (area under the curve=0.624).3) The total amount of moesin in PBMC was significantly higher in CTD-PAH patients (P=0.033). Surface expression of moesin was correlated with serum anti-moesin antibody (P=0.045, r=0.564).4) In SLE-PAH, endothelin-1outperformed other biomarkers in receiver operating characteristic analysis (AUC=0.704).[Conclusion]1) These results suggested that RDW served as a prognostic indicator in CTD-PAH.2) Anti-moesin antibody and endothelin-1may become diagnostic biomarkers for CTD-PAH and SLE-PAH respectively.3) There were indications that the serum level of anti-moesin antibody was raised in CTD-PAH partly as a result of the overexpression of moesin on the surface of PBMC.