Clinical characteristics and survival of patients with three major connective tissue diseases associated with pulmonary hypertension: A study from China.

The present cross-sectional study investigated the clinical characteristics and survival of patients with three types of connective tissue disease associated with pulmonary hypertension (CTD-PH) diagnosed early by echocardiography. A total of 218 patients with CTD-PH were included in the present study. Patients with the three major types of CTD, namely systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and primary Sjögren's syndrome (pSS), were included. PH was diagnosed based on pulmonary arterial systolic pressure >35 mmHg, as measured by Doppler echocardiography. Demographic data, clinical features, laboratory results and echocardiographic parameters were collected and analyzed. The Kaplan-Meier method was used to calculate survival rates. Multivariate analysis was used to identify independent factors affecting mortality. Compared with patients with CTD with pSS (6.5%) or SLE (3.8%), those with SSc had a higher prevalance of PH (12.9%). Patients with SSc-PH had the highest rate of lung involvement (81.2%) and 42.2% of patients were classified as World Health Organization-function class III/IV at the time of diagnosis with PH. The overall survival rate among patients with CTD-PH at 1, 3 and 5 years was 81.4, 72.4 and 56.9%, respectively. Patients with SLE-PH appeared to have the most favorable prognosis and patients with SSc-PH had the poorest relative outcomes. Multivariate analysis revealed that age ≥50 years was the only independent risk factor for mortality. In conclusion, among the patients with CTDs investigated, the prevalence of PH was highest among those with SSc. Patients with SSc-PH had the highest prevalence of pulmonary involvement, the lowest survival rate and the worst prognosis.