Robert P Baughman1, Oksana A Shlobin2, Athol U Wells3, Esam H Alhamad4, Daniel A Culver5, Joseph Barney6, Francis C Cordova7, Eva M Carmona8, Mary Beth Scholand9, Marlies Wijsenbeek10, Sivagini Ganesh11, Surinder S Birring12, Vasilis Kouranos3, Lanier O'Hare6, Joanne M Baran5, Joseph G Cal4, Elyse E Lower13, Peter J Engel14, Steven D Nathan2. 1. University of Cincinnati Medical Center, 1001 Holmes Building, 200 Albert Sabin Way, Cincinnati, OH 45267, USA. Electronic address: baughmrp@ucmail.uc.edu. 2. INOVA Medical Center, Fairfax, VA, USA. 3. Royal Hospital Brompton, London, UK. 4. Department of Medicine, Division of Pulmonary Medicine, King Saud University, Riyadh, Saudi Arabia. 5. Cleveland Clinic, Cleveland OH, USA. 6. University of Alabama, Birmingham, AL, USA. 7. Temple University, Philadelphia PA, USA. 8. Mayo Clinic, Rochester Minn, USA. 9. University of Utah, Salt Lake City, UT, USA. 10. Department of Respiratory Medicine, Erasmus University Medical Centre, Rotterdam, NL, Netherlands. 11. University of Southern California, Los Angeles, CA, USA. 12. King's College, London, UK. 13. University of Cincinnati Medical Center, 1001 Holmes Building, 200 Albert Sabin Way, Cincinnati, OH 45267, USA. 14. The Christ Hospital, Cincinnati, OH, USA.
Abstract
BACKGROUND: Pulmonary hypertension (PH) is a significant cause of morbidity and mortality in sarcoidosis. We established a multi-national registry of sarcoidosis associated PH (SAPH) patients. METHODS: Sarcoidosis 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). RESULTS: A 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. CONCLUSIONS: The 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.
BACKGROUND:Pulmonary hypertension (PH) is a significant cause of morbidity and mortality in sarcoidosis. We established a multi-national registry of sarcoidosis associated PH (SAPH) patients. METHODS:Sarcoidosispatients 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). RESULTS: A 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. CONCLUSIONS: The 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.
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