Characteristics of connective tissue disease-associated pulmonary arterial hypertension: a retrospective cohort study

Background and aim: Connective tissue diseases (CTD) often presents with pulmonary arterial hypertension (PAH). This study aimed to evaluate the clinical characteristics of patients with CTD-associated PAH (CTD-PAH). Methods: A retrospective cohort study was conducted at the PAH center in the Western China. Fifty-four consecutive adult patients with a diagnosis of CTD-PAH confirmed by right heart catheterization during the previous 3 years were enrolled. Patients' baseline characteristics were evaluated and log-rank test was applied to determine the predictors of all-cause mortality. Results: Fifty-four patients with CTD-PAH were included and followed for 46 months, of which 48.1% experienced pericardial effusion. Although univariate Cox regression analysis suggested that poor WHO-FCS (III and IV), lower cardiac index (<2.5 L/min/m(2)) and the presence of pericardial effusion at baseline were significantly associated with all-cause death, pericardial effusion was the only independent predictor of mortality (HR=3.74, 95% CI=1.48-10.8, p=0.03). Moreover, the Log-rank tests indicated that patients with SLE-PAH had worse survival rates than those with PAH associated with other CTDs (p=0.001); while CTD patients with interstitial lung diseases (ILD) associated with PAH had worse survival than CTD-PAH patients without ILD. Conclusions: CTD-PAH is a severe clinical syndrome associated with high mortality. In these patients, pericardial effusion was an independent predictor of mortality. Patients with SLE-PAH or ILD also had poor clinical outcomes.