Idiopathic PAH vs Scleroderma-Associated PAH: Pulmonary Function and Hemodynamic Differences

This article originally appeared here.
Share this content:
People with SSc-PAH had much higher levels of brain natriuretic peptide and N-terminal pro B-type naturiuretic peptide vs those with iPAH.
People with SSc-PAH had much higher levels of brain natriuretic peptide and N-terminal pro B-type naturiuretic peptide vs those with iPAH.

SAN DIEGO — Patients with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) display important differences in hemodynamic and pulmonary function parameters compared with patients with idiopathic PAH (iPAH), according to a study presented at the American College of Rheumatology 2017 Annual Meeting, held November 3-8 in San Diego, California.

Researchers from the Cleveland Clinic conducted a retrospective chart review to identify characteristics specific to patients with SSc-PAH and iPAH. Using data from the Cleveland Clinic Pulmonary Hypertension Database, the study sampled a total of 862 patients: 686 with iPAH and 176 with SSc-PAH. Thirty demographic, laboratory, and hemodynamic variables were examined.

The results show statistically significant differences between SSc-PAH groups and iPAH groups for a set of variables that include age (P <.001), sex (P <.001), forced vital capacity percentage (P =.002), total lung capacity percentage (P <.001), systolic blood pressure (P =.018), mortality status (P =.016), 6-minute walk distance (P <.001), right ventricular systolic pressure (P =.012), mean pulmonary artery pressure (P <.001), pulmonary vascular resistance (P =.002), forced expiratory volume in 1 second to forced vital capacity percent ratio (P <.001), and transfer factor (P <.001).

The researchers note that patients with SSc-PAH had much higher levels of brain natriuretic peptide and N-terminal pro B-type natriuretic peptide compared with patients with iPAH, and similar hemodynamic parameters (P <.001 and P =.013, respectively). "Further research will be necessary to help delineate other relevant factors that may aid in caring for these patients," they concluded.

Reference

Hannan A, Dweik R, Highland KB, et al. Analysis of prognostic determinants of scleroderma-associated pulmonary arterial hypertension and idiopathic pulmonary arterial hypertension. Presented at: 2017 American College of Rheumatology 2017 Annual Meeting; November 3-8, 2017; San Diego, CA. Poster 2680.

Sign Up for Free e-newsletters