The change of lung diffusion capacity does not influence the prognosis in patients with pulmonary arterial hypertension associated with systemic sclerosis

Pulmonary arterial hypertension, associated with systemic sclerosis (PAH-SSc)-devastating disease with high mortality. The decrease of lung diffusion capacity (DLCO) is often in patients with PAH-SSc, but influence of the change of DLCO on survival are not well described. Aim: In this study, we aimed to define whether change influences on survival in patients with PAH-SSc. Methods: We performed a cohort study of PAH-SSc patients (n=76; mean age 53±11 years, 92% female) evaluated at the Institute. The Kaplan-Meier method and Cox regression analysis were used to assess survival and predictors of mortality. The clinical characteristics and survival were compared in patients categorized by PAH severity based on the World Health Organization classification and 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Results: We found that survival to be dependent on the initial DLCO value. On ROC curve analysis, DLCO Conclusion: Only DLCO level less than 43,8% is a predictor of mortality and should be used to risk-stratify in PAH-SSc patients.