Factors Influencing Improved Survival in Systemic Sclerosis-Associated PAH

Pulmonary arterial hypertension with systemic sclerosis associated-survival improved over time among patients aged 70 years or younger, but not among the elderly.

Between 2006 and 2017, systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) survival improved over time among patients aged ≤70 years, but not among the elderly, according to study results published in CHEST.

PAH occurs in approximately 10% of patients with SSc and is a leading cause of death, with a 3-year survival rate of only 52%. While the combination of regular screening for PAH in SSc and a strategy of combination therapy may have improved survival in these patients in the last 10 years, no data are available to demonstrate this association.

Therefore, researchers in France used a multivariate Cox regression model adjusted for clinically relevant baseline confounders to assess the association between the occurrence of death and date of PAH diagnosis to compare 2 periods of the same duration (2006-2011 vs 2012-2017). They did not observe a significant difference in survival between PAH patients diagnosed between 2012 and 2017 compared with 2006 and 2011 (P =.29). However, they did observe a significant increase in survival among patients aged ≤70 years between 2006 and 2011 and 2012 and 2017 (P =.046) but not among older patients (P =.44). In addition, a significantly higher percentage of initial endothelin receptor antagonist/phosphodiesterase type-5 inhibitor combination therapy was observed in younger patients diagnosed between 2012 and 2017 vs 2006 and 2011 (42.9% vs 19.5%; P =.002) but not in older patients.

“A hypothesis that could explain this finding is that younger patients have less severe disease, and changes in survival over time may be related to severity of disease,” the researchers wrote. “However, we did not observe any significant interaction between the two study periods and baseline variables including the common poor prognostic factors.”

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The researchers noted that further investigations are needed to determine whether the survival rates are related to the increase in initial combination therapy and/or improvements in medical care in general.  

Disclosure: Several study authors declared affiliations with the pharmaceutical industry. Please see the original reference for a full list of authors’ disclosures.


Hachulla E, Launay D, Boucly A, et al. Survival improved in systemic sclerosis associated pulmonary arterial hypertension patients aged 70 years or less over the period 2006-2017 in France [published online October 19, 2019]. CHEST. doi:10.1016/j.chest.2019.10.045