Abstract

Despite efforts at early detection, systemic sclerosis pulmonary hypertension (SSc-PH) patients present with advanced disease. We sought to determine whether endothelial biomarkers (asymmetric dimethylarginine [ADMA], soluble endoglin [sEng], and pentraxin-3 [PTX-3]) can determine SSc-PH risk or differentiate between SSc-PH subgroups.ADMA, sEng, and PTX-3 were measured by ELISA in four groups: 1) 18 healthy controls; 2) 74 SSc-PH patients; 3) 44 patients with high-risk for PH features; 4) 10 patients with low-risk for PH features. High-risk features included a diffusion capacity (DLCO) <55% with forced vital capacity (FVC) >70%, or an FVC/DLCO ratio of >1.6, or a right ventricular systolic pressure on echocardiogram =40mmHg. ADMA, sEng, and PTX-3 were compared between these four groups as well as stratified based on the three SSc-PH clinical classification groups (pulmonary arterial hypertension [PAH], left-heart disease [LHD], interstitial lung disease [ILD]).PTX-3 was significantly lower in SSc subjects at low risk for PH [median 27.0pg/mL [IQR 19.0, 47.3, p<0.003] than the other groups. The area under the receiving operator characteristic curve was 0.87 (95% CI 0.76-0.98, p=0.0002) to differentiate low-risk from high-risk for PH patients. PTX-3 was significantly lower in SSc-PH from LHD (57.5pg/mL [39.8, 79.0], p<0.01) compared to either SSc-PH from PAH (85.5pg/mL [56.3, 104.5]) or ILD (90.3pg/mL [74.9, 111.0]). Neither ADMA nor sEng differed between the four groups.Pentraxin-3 is a promising biomarker of PH risk status in SSc patients as well as a possible marker of pre-capillary pulmonary hypertension, which should be validated in an external cohort.

View details for DOI 10.1002/acr.25180

View details for PubMedID 37365746