Systemic Sclerosis Increases Mortality in PAH Patients, Chinese Study Says

A cohort study with Chinese patients identified systemic sclerosis (SSc) as the key underlying disease that significantly worsens the clinical outcomes of patients with pulmonary arterial hypertension (PAH).

The study “Clinical characteristics and survival of pulmonary arterial hypertension associated with three major connective tissue diseases: A cohort study in China” appeared in the International Journal of Cardiology.

PAH is a severe complication and key factor leading to the death of patients with connective tissue diseases (CTDs) such as SSc and systemic lupus erythematosus (SLE).

Now, researchers investigated clinical characteristics and survival in a well-defined population of Chinese patients with CTD-PAH. Their study included 190 PAH patients with SLE, SSc or primary Sjögren’s syndrome (pSS); their mean age was 37.8 years.

Researchers defined the type of CTD at the time of enrollment for the study, using criteria defined for each disease: SLE was diagnosed according to the American College of Rheumatology (ACR) criteria revised in 1997; limited or diffuse cutaneous SSc was diagnosed according to the American Rheumatism Association criteria established in 1980 and confirmed by the 2013 ACR/European League Against Rheumatism (EULAR) classification; and pSS was diagnosed according to revised criteria proposed by the American-European Consensus Group in 2002.

Researchers analyzed several parameters, including demographic data, CTD classification criteria, patients’ clinical characteristics, treatments, and the results of various lab tests such as blood counts and levels of hepatic function enzymes.

The results showed that 58.4 percent of the patients with PAH had SLE as an underlying CTD, while only 26.3 percent had SSc and 15.3 percent pSS. Patients with SLE showed the most favorable prognosis; one-year and three-year survival rates for SLE-PAH patients were 94.1 and 81.3 percent respectively, buttony 72.5 and 63.6 percent for SSc-PAH patients. As such, the authors found that the type of CTD is a novel, independent predictor of mortality in patients with CTD-PAH.

Overall, this “is the first to describe outcomes of patients with pSS-PAH and confirmed that patients with SSc-PAH have the worst prognosis among PAH patients with CTDs,” the researchers concluded. “Further studies are needed to determine appropriate therapeutic strategies for patients with different underlying CTDs.”