Validation of noninvasive screening methods for pulmonary arterial hypertension in patients with systemic sclerosis

Pulmonary arterial hypertension (PAH) is a life-threatening condition that leads to early disability and death. As one of the complications of systemic sclerosis (SSc), it is important to develop screening models to improve diagnosis of PAH in these patients. Aim: To test recommended PAH screening models in a Russian population of SSc patients to identify those at high risk for PAH using both previous and current hemodynamic definitions of PAH. Material and methods. The study included 138 patients with SSc who underwent a one-stage clinical, laboratory, and instrumental examination at the Institute of Rheumatology between November 2011 and November 2019. We analyzed the sensitivity, specificity, positive and negative predictive values of two screening models (algorithm DETECT and EchoCG screening), according to two definitions of PH and PAH. Results . The sensitivity of the algorithm DETECT in identifying patients at high risk of PAH according to the 2022 definition was lower (99%) than the 2016 definition (100%), while the specificity remained the same (24%). When using EchoCG, the sensitivity was lower than the detection algorithm (95% for the new definition, 96% for the old definition), but the specificity was higher (38% for both definitions). Conclusions . The effectiveness of the two screening models appears to be equivalent, although their low specificity raises concern. Further research is needed to identify new markers or to narrow the target population for the EchoCG screening test for PAH in SJS.