• Katarina Zeder, Alexander Avian, Gerhard Bachmaier, Philipp Douschan, Vasile Foris, Teresa Sassmann, Florentine C Moazedi-Fuerst, Winfried B Graninger, Franz Hafner, Marianne Brodmann, Wolfgang Salmhofer, Horst Olschewski, and Gabor Kovacs.
• Ludwig Boltzmann Institute for Lung Vascular Research, Graz, Austria; Division of Pulmonology, Department of Internal Medicine, Medical University of Graz, Graz, Austria.
• Chest. 2021 Feb 1; 159 (2): 781-790.

BackgroundPulmonary hemodynamics during exercise may reveal early pulmonary vascular disease and may be of clinical and prognostic relevance in systemic sclerosis (SSc). We aimed to assess the prognostic relevance of exercise pulmonary resistances in patients with SSc with no or mildly increased mean pulmonary arterial pressure (mPAP).Research QuestionAre pulmonary resistances at peak exercise independent predictors of mortality in systemic sclerosis?Study Design And MethodsAll SSc patients with resting mPAP < 25 mm Hg and at least one year of follow-up data who underwent symptom-limited exercise right heart catheterization between April 2005 and December 2018 were analyzed retrospectively. Age-adjusted Cox regression analysis was used to evaluate the association between pulmonary resistances and all-cause mortality.ResultsThe cohort consisted of 80 patients: 73 women and 7 men with a mean age of 57 years (interquartile range [IQR], 47-67 years) and a mean follow-up time of 10.4 years (IQR, 8.5-11.8 years). At baseline, resting mPAP of ≤ 20 mm Hg and 21 to 24 mm Hg was found in 68 and 12 patients, respectively. Pulmonary vascular resistance (PVR) and total pulmonary resistance (TPR) at peak exercise were associated significantly with mortality (P = .006 [hazard ratio (HR), 2.20; 95% CI, 1.26-3.87] and P = .026 [HR, 1.56; 95% CI, 1.06-2.29]), whereas resting PVR and TPR were not (P = .087 [HR, 2.27; 95% CI, 0.89-5.83] and P = .079 [HR, 1.88; 95% CI, 0.93-3.80]). The mPAP per cardiac output (CO) and transpulmonary gradient (TPG) per CO slopes were associated significantly with mortality (P = .047 [HR, 1.14; 95% CI, 1.002-1.286] and P = .034 [HR, 1.34; 95% CI, 1.02-1.76]) as well. The area under the receiver operating characteristic curve for exercise PVR to predict 10-year mortality was 0.917 (95% CI, 0.797-1.000).InterpretationPVR and TPR at peak exercise, mPAP/CO slope, and TPG/CO slope are predictors of age-adjusted long-term mortality in SSc patients with no or mildly increased pulmonary arterial pressure.Copyright © 2020 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

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