Distance assessment of the risk of pulmonary hypertension associated with connective tissue diseases: do biomarkers help?

Abstract

Proposed PAH risk assessment models require face-to-face assessment.

The aim of the study to create a model for distance evaluation.

The study included 97 patients, mean age 50(39,58) years, with a duration of PAH-therapy 44 months. During the follow-up, 41 patients died.

Cox models revealed the effect of biomarkers on survival: uric acid(UA) levels 435 mmol/l (HR4.09[1.82;9.23]), CRP levels (HR1.04[1.02;1.06]) and the presence of anticentromeric antibodies (AA) (HR2.89[1.35;6,21]). A method for rapid assessment of the risk of death in patients with PAH-CTD, comprising determining biomarkers, characterized in that the biomarker is a UA level (X1), a concentration of CRP (X2) and the presence of AA (X3); X1 and X2 are evaluated in points, namely: at a value of X1≥435 μmol/l, "1 point" is assigned, at a value of <435 μmol/l, "0 points" are assigned, in the absence of X3, "0 points" are assigned, in the presence of X3-"1 point"; X2-in absolute values, g/l; based on the obtained data, the probability of adverse outcome of PAH-CTD (p) is determined by the formula:p = 0.1624×exp(1.4103×X1+0.0365×X2+1.0636×X3), if p<0.5-low risk of outcome is determined, if 0,5≤p<0,7-mean risk is determined, in cases of p≥0,7-high risk of PAH-CTD is determined. The ROC analysis showed an AUC of 0.757(95%CI 0.656;0.857), p<0.0001. For a risk value of 0.5, sensitivity-88%, specificity-56%. For a risk value of 0.7, sensitivity-68%, specificity-67%. The risk of death was determined using Cox regression, depending on the risk calculated with our model. It was 2.85 (1.81 - 4.50), which is comparable to known prognosis markers - ESC/ERS 2,65 (1,76 – 4,00) and REVEAL 2,89 (1,72 – 4,89), p<0,001.