Clinical and laboratory characteristics of systemic sclerosis patients with pulmonary arterial hypertension in China.

OBJECTIVES: Pulmonary arterial hypertension (PAH) is associated with significant morbidity and mortality, especially in systemic sclerosis (SSc). Since there was no published study regarding PAH in the Chinese SSc population, we aimed to describe a cohort to provide some data for early diagnosis.

METHODS: We evaluated 236 systemic sclerosis patients prospectively registered in the EUSTAR (European League Against Rheumatism Scleroderma Trial and Research Group) database from the Peking Union Medical College Hospital from 2009 to 2012. Among them, 33 individuals received right heart catheterisations (RHC) while the remaining patients were grouped by echocardiographic data. These patients were classified into two groups, PAH and non-PAH group. Their clinical and laboratory features were statistically analysed to identify possible risk factors for PAH in Chinese SSc population.

RESULTS: The possible prevalence of PAH in SSc patients was approximately 11% in our study. Digital ulcers (52.0% vs. 31.2%), telangiectasias (64.0% vs. 37.6%) and gastroesophageal reflux disease (GERD) (60.0% vs. 36.2%) were more common in SSc patients with PAH. Some laboratory results were also proved to be significantly correlated with it. Logistic regression analysis showed that telangiectasias (OR=2.888, 95% CI=1.176-7.093), presence of GERD (OR=2.592, 95% CI=1.067-6.296), anti-RNP positivity (OR=24.384, 95% CI=1.978-36.651), IgA level elevation (OR=8.745, 95% CI 4.838-122.896) and FVC/TLCO ratio (OR=97.067, 95% CI 12.475-755.271) were associated with an increased odds for PAH in SSc patients.

CONCLUSIONS: This study described possible predictors of PAH in Chinese SSc population, which have been supported by similar studies in other ethnic groups.