Assessment of Life Expectancy in Scleroderma-Associated Pulmonary Arterial Hypertension

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Although patients with SSc-ILD-PH had a longer life expectancy than those with SSc-PAH, there was no significant survival difference between the 2 groups.
Although patients with SSc-ILD-PH had a longer life expectancy than those with SSc-PAH, there was no significant survival difference between the 2 groups.
The following article features coverage from ACR 2017 in San Diego, California. Click here to read more of Rheumatology Advisor's conference coverage.

SAN DIEGO – Patients with scleroderma-associated isolated pulmonary arterial hypertension (SSc-PAH) have a slightly higher mortality risk than patients with scleroderma-associated pulmonary hypertension with concomitant interstitial lung disease (SSc-ILD-PH), and significant differences exist between the 2 patient populations with regard to key cardiovascular markers, according to a study presented at the ACR/ARHP 2017 Annual Meeting, held November 3-8.

“Relatively little data exist in the literature to characterize the differences between patients with SSc-PAH and SSc-ILDPH,” said the investigators of this trial. “Our aim was to compare the characteristics of SSc-PAH and SSc-ILD-PH patients from the Cleveland Clinic Pulmonary Hypertension Database to help define differences that may exist between these 2 groups.”  They therefore conducted a retrospective review of patients with SSc-ILD-PH (n=21) and SSc-PAH (n=155) to examine differences in hemodynamic, demographic, and laboratory data.

Age (64.5±11.3 vs 58.0±14.7; P =.019), pulmonary vascular resistance (8.4±5.3 vs 5.2±3.8; P =.013), cardiac index by thermodilution (2.5±0.76 vs 2.9±0.90; P =.036), percent-estimated forced vital capacity (71.2±21.0 vs 60.6±14.2; P =.027), and NT-proBNP levels (1485.5 vs 187.0; P =.002) were found to be significantly different between SSc-PAH and SSc-ILD-PH groups, respectively.

The researchers also found that patients with SSc-ILD-PH had a longer life expectancy than those with SSc-PAH (estimated median time to death: 13.19 years [95% CI: 3.79- 20.72] vs 4.89 years [95% CI: 4.01-6.98], respectively); however, there was no significant difference for survival between the 2 groups (P =.1743). Considering patients with SSc-PAH were older, these findings are somewhat limited.

In addition to the effect of age on survival in this cohort, the investigators suggest that the observed longer survival among “SSc-ILD-PH patients may be explained by some lurking variables that were not available for analysis, such as autoantibody subsets.”

Visit Rheumatology Advisor's conference section for continuous coverage from ACR 2017.

Reference

Hannan A, Dweik R, Highland KB, et al. Comparison of scleroderma associated isolated pulmonary arterial hypertension and pulmonary hypertension with concomitant interstitial lung disease. Presented at: ACR/ARHP 2017 Annual Meeting; November 3-8, 2017; San Diego, California; Abstract 2678.

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