Assessment of Life Expectancy in Scleroderma-Associated Pulmonary Arterial Hypertension

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.”

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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.