• Robert P Baughman, Oksana A Shlobin, Athol U Wells, Esam H Alhamad, Daniel A Culver, Joseph Barney, Francis C Cordova, Eva M Carmona, Mary Beth Scholand, Marlies Wijsenbeek, Sivagini Ganesh, Surinder S Birring, Vasilis Kouranos, Lanier O'Hare, Joanne M Baran, Joseph G Cal, Elyse E Lower, Peter J Engel, and Steven D Nathan.
• University of Cincinnati Medical Center, 1001 Holmes Building, 200 Albert Sabin Way, Cincinnati, OH 45267, USA. Electronic address: baughmrp@ucmail.uc.edu.
• Respir Med. 2018 Jun 1; 139: 72-78.

BackgroundPulmonary hypertension (PH) is a significant cause of morbidity and mortality in sarcoidosis. We established a multi-national registry of sarcoidosis associated PH (SAPH) patients.MethodsSarcoidosis patients with PH confirmed by right heart catheterization (RHC) were studied. Patients with pulmonary artery wedge pressure (PAWP) of 15 mmHg or less and a mean pulmonary artery pressure (mPAP) ≥ 25 Hg were subsequently analyzed. Data collected included hemodynamics, forced vital capacity (FVC), diffusion capacity of carbon monoxide (DLCO), chest x-ray, and 6-min walk distance (6MWD).ResultsA total of 176 patients were analyzed. This included 84 (48%) cases identified within a year of entry into the registry and 94 (53%) with moderate to severe PH. There was a significant correlation between DLCO percent predicted (% pred) andmPAP (Rho = -0.228, p = 0.0068) and pulmonary vascular resistance (PVR) (Rho = -0.362, p < 0.0001). PVR was significantly higher in stage 4 disease than in stage 0 or 1 disease (p < 0.05 for both comparisons). About two-thirds of the SAPH patients came from the United States (US). There was a significant difference in the rate of treatment between US (67.5%) versus non-US (86%) (Chi Square 11.26, p = 0.0008) sites.ConclusionsThe clinical features of SAPH were similar across multiple centers in the US, Europe, and the Middle East. The severity of SAPH was related to reduced DLCO. There were treatment differences between the US and non-US centers.Copyright © 2018. Published by Elsevier Ltd.

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