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

Blood Vessels Lungs PAH
Blood Vessels Lungs PAH
Results show statistically significant differences between SSc-PAH groups and iPAH groups for variables including age, sex, FVC capacity, total lung capacity, and FEV1, among others.

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.

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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.

This article originally appeared on Rheumatology Advisor