Clinical and laboratory profiles of 136 systemic sclerosis patients with and without echocardiographically detected pulmonary hypertension

Aim This study aims to review the clinical and laboratory profiles of systemic sclerosis (SSc) patients with and without echocardiographically detected pulmonary hypertension (PH) in China. Patients and methods The study included 136 consecutive patients treated from 1992 to 2012. Diagnosis of SSc was made according to the 1980 revision of the American College of Rheumatology SSc criteria. PH was defined as systolic pulmonary artery pressure ≥ 40 mmHg detected by Doppler echocardiography. The clinical and laboratory parameters of SSc patients with pulmonary hypertension (SSc-PH) were compared to those of SSc patients without pulmonary hypertension (SSc-no PH). Results Of the 136 SSc patients, 28 (20.6 %) were diagnosed as having PH by echocardiography. Upon comparison with the SSc-no PH patients, SSc-PH patients were observed to have a significantly higher frequency of subjective dyspnea (P = 0.010) and a higher rate of anti-nuclear RNP (anti-nRNP) antibody positivity (P = 0.028). We also observed that the percentage of SSc-PH patients with increased urea nitrogen is significantly higher than that of SSc-no PH patients after correction for multiple testing (P = 0.006, compared to patients with normal values). Conclusion This study demonstrates that SSc patients with PH detected by echocardiography had characteristic clinical and laboratory features. More specific treatment addressing these aspects should be offered to improve the curative effect of therapy in SSc-PH patients.