Clinical features of sarcoidosis associated pulmonary hypertension: Results of a multi-national registry

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; Steven D. Nathan

doi:10.1016/j.rmed.2018.04.015

Clinical features of sarcoidosis associated pulmonary hypertension: Results of a multi-national registry

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, Steven D. Nathan

Pulmonary and Critical Care Medicine

Research output: Contribution to journal › Article › peer-review

21 Scopus citations

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 (DL_CO), 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 DL_CO 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.

Original language	English (US)
Pages (from-to)	72-78
Number of pages	7
Journal	Respiratory Medicine
Volume	139
DOIs	https://doi.org/10.1016/j.rmed.2018.04.015
State	Published - Jun 2018

Keywords

Epidemiology
Sarcoidosis
Sarcoidosis associated pulmonary hypertension

ASJC Scopus subject areas

Pulmonary and Respiratory Medicine

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10.1016/j.rmed.2018.04.015

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Baughman, R. P., Shlobin, O. A., Wells, A. U., Alhamad, E. H., Culver, D. A., Barney, J., Cordova, F. C., Carmona, E. M., Scholand, M. B., Wijsenbeek, M., Ganesh, S., Birring, S. S., Kouranos, V., O'Hare, L., Baran, J. M., Cal, J. G., Lower, E. E., Engel, P. J., & Nathan, S. D. (2018). Clinical features of sarcoidosis associated pulmonary hypertension: Results of a multi-national registry. Respiratory Medicine, 139, 72-78. https://doi.org/10.1016/j.rmed.2018.04.015

Baughman, RP, Shlobin, OA, Wells, AU, Alhamad, EH, Culver, DA, Barney, J, Cordova, FC, Carmona, EM, Scholand, MB, Wijsenbeek, M, Ganesh, S, Birring, SS, Kouranos, V, O'Hare, L, Baran, JM, Cal, JG, Lower, EE, Engel, PJ & Nathan, SD 2018, 'Clinical features of sarcoidosis associated pulmonary hypertension: Results of a multi-national registry', Respiratory Medicine, vol. 139, pp. 72-78. https://doi.org/10.1016/j.rmed.2018.04.015

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title = "Clinical features of sarcoidosis associated pulmonary hypertension: Results of a multi-national registry",

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.",

keywords = "Epidemiology, Sarcoidosis, Sarcoidosis associated pulmonary hypertension",

author = "Baughman, {Robert P.} and Shlobin, {Oksana A.} and Wells, {Athol U.} and Alhamad, {Esam H.} and Culver, {Daniel A.} and Joseph Barney and Cordova, {Francis C.} and Carmona, {Eva M.} and Scholand, {Mary Beth} and Marlies Wijsenbeek and Sivagini Ganesh and Birring, {Surinder S.} and Vasilis Kouranos and Lanier O'Hare and Baran, {Joanne M.} and Cal, {Joseph G.} and Lower, {Elyse E.} and Engel, {Peter J.} and Nathan, {Steven D.}",

note = "Publisher Copyright: {\textcopyright} 2018",

year = "2018",

month = jun,

doi = "10.1016/j.rmed.2018.04.015",

language = "English (US)",

volume = "139",

pages = "72--78",

journal = "Respiratory Medicine",

issn = "0954-6111",

publisher = "W.B. Saunders Ltd",

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TY - JOUR

T1 - Clinical features of sarcoidosis associated pulmonary hypertension

T2 - Results of a multi-national registry

AU - Baughman, Robert P.

AU - Shlobin, Oksana A.

AU - Wells, Athol U.

AU - Alhamad, Esam H.

AU - Culver, Daniel A.

AU - Barney, Joseph

AU - Cordova, Francis C.

AU - Carmona, Eva M.

AU - Scholand, Mary Beth

AU - Wijsenbeek, Marlies

AU - Ganesh, Sivagini

AU - Birring, Surinder S.

AU - Kouranos, Vasilis

AU - O'Hare, Lanier

AU - Baran, Joanne M.

AU - Cal, Joseph G.

AU - Lower, Elyse E.

AU - Engel, Peter J.

AU - Nathan, Steven D.

PY - 2018/6

Y1 - 2018/6

N2 - 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.

AB - 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.

KW - Epidemiology

KW - Sarcoidosis

KW - Sarcoidosis associated pulmonary hypertension

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UR - http://www.scopus.com/inward/citedby.url?scp=85046834821&partnerID=8YFLogxK

U2 - 10.1016/j.rmed.2018.04.015

DO - 10.1016/j.rmed.2018.04.015

M3 - Article

C2 - 29858005

AN - SCOPUS:85046834821

SN - 0954-6111

VL - 139

SP - 72

EP - 78

JO - Respiratory Medicine

JF - Respiratory Medicine

ER -

Clinical features of sarcoidosis associated pulmonary hypertension: Results of a multi-national registry

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