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

Abstract

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.

The diagnostic and prognostic value of growth differentiation factor‐15 in systemic lupus erythematosus‐associated pulmonary arterial hypertension

“…PAH is a common and severe complication of SLE. 18 Noninvasive biomarkers are needed to help identify and risk‐stratify SLE‐PAH patients. In our study, we confirmed GDF‐15 as a useful biomarker of SLE‐PAH prognosis.…”

“…PAH is a common and severe complication of SLE 18 . Noninvasive biomarkers are needed to help identify and risk‐stratify SLE‐PAH patients.…”

“…PAH is a common and severe complication of SLE. 18 Noninvasive biomarkers are needed to help identify and risk-stratify SLE-PAH patients. In our study, we confirmed GDF-15 as a useful biomarker of SLE-PAH T A B L E 2 Univariable and multivariable analysis of associations between GDF-15 with death and TGA.…”

Survival and prognosis analysis of systemic lupus erythematosus patients with pulmonary hypertension: A systematic review and meta-analysis

“…All of them were reviewed in full text to determine eligibility based on inclusion criteria. As such, 21 studies, [5,[10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29] involving 19 of them were included in the survival-relevant meta-analysis and 12 were included to analyze prognostic factors (Fig. 1).…”

Association between systemic lupus erythematosus and pulmonary hypertension: Evidence from Meta-Analysis and Mendelian Randomization

“…The discrepancies were chie y put down to the different diagnostic methods of transthoracic echocardiogram (TTE) and right heart catheterization (RHC). Other investigators estimated the prevalence of PH to be 2.7-17.5% based on TTE of SLE patients [8,9], while the prevalence of PH was 0.5-9.3% when diagnosed by RHC [10]. A meta-analysis in 2014 showed the prevalence of PAH in SLE patients among the Chinese population to be 1.8-23.3% [11], and the latest meta-analysis in 2018 showed a prevalence of 8% [12].…”