Systemic sclerosis associated with poor survival in CTD-PAH
medwireNews: A Chinese cohort study identifies factors associated with prognosis among patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH).
Of 190 patients with CTD-PAH who visited the researchers’ referral center from 2006 to 2014, 58.4% had systemic lupus erythematosus (SLE) as the underlying CTD, whereas 26.3% had systemic sclerosis (SSc), and 15.3% had primary Sjögren's syndrome (pSS).
Patients with SLE-PAH were younger than those with SSc-PAH and pSS-PAH, with mean ages of 34.6 years, 43.3 years, and 40.6 years, respectively. The majority (≥90%) of patients in all three groups were women, report Xiaofeng Zeng (Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing) and study co-authors.
The researchers found that cumulative survival “differed markedly” for patients with the three different CTDs; 1-year survival rates were 94.1% for SLE-PAH, 72.5% for SSc-PAH, and 78.5% for pSS-PAH. The corresponding 3- year rates were 81.3%, 63.3%, and 72.9%, and 5-year rates were 61.0%, 43.9%, and 64.8%.
In a multivariate analysis accounting for factors including age, gender, disease duration, cardiac function, and treatment, patients who had SSc as the underlying CTD had a significantly greater risk for mortality than those with the other CTDs, with a hazard ratio (HR) of 1.68.
Those with a 6-minute walk distance (6MWD) of 380 m or less were also at significantly increased mortality risk than those with longer 6MWD (HR=3.22).
These findings “confirmed that patients with SSc-PAH have the worst prognosis among PAH patients with CTDs,” the researchers write in the International Journal of Cardiology.
However, they note that “there is still no clear explanation for the difference in survival between SSc-, SLE- and pSS-associated PAH.”
Zeng and colleagues caution that the majority of patients in their study had severe PAH, “which is associated with a much higher mortality rate than that of patients identified earlier in the course of the disease.”
“An early detection strategy is needed to identify patients in the early stages of PAH,” they add.
And the team concludes: “Further studies are needed to determine appropriate therapeutic strategies for patients with different underlying CTDs.”
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