TY - JOUR
T1 - High-risk sarcoidosis
T2 - Current concepts and research imperatives
AU - Sauer, William H.
AU - Stern, Barney J.
AU - Baughman, Robert P.
AU - Culver, Daniel A.
AU - Royal, Walter
N1 - Funding Information:
The authors thank their patients; study participants who have enrolled in sarcoidosis-related research studies; the many organizations that have supported the sarcoidosis community and its research mission; Drs. Jerry Eu, George Mensah, Lora Reineck, and Antonello Punturieri, and the National Heart, Lung, and Blood Institute; and other Institutes/Centers of the National Institutes of Health for the support of this workshop. They also thank the remainder of the workshop participants for thoughtful discussions that led to the development of these recommendations and Megan Marchant for assistance with editing and formatting this manuscript.
Publisher Copyright:
Copyright © 2017 by the American Thoracic Society.
PY - 2017/12
Y1 - 2017/12
N2 - Sarcoidosis is a disease with heterogeneous manifestations and outcomes, varying in part on the basis of organ involvement. Specifically, patients with sarcoidosis at risk for poor outcomes include individuals with treatment-resistant pulmonary sarcoidosis, including fibrotic pulmonary disease and pulmonary hypertension, as well as those with cardiac, neurologic, and multiorgan disease. The limited but available data relating to these patients with high-risk sarcoidosis, defined as those patients with presentations requiring medical intervention to avoid progressive disability or premature death, was evaluated as part of the National Heart, Lung, and Blood Institute's workshop to improve understanding of these disease manifestations. In particular, knowledge gaps that preclude a greater understanding of the pathogenesis and management of these severe sarcoidosis clinical phenotypes were identified in the workshop. Research strategies are proposed to address critical knowledge gaps that would further our understanding of these disease manifestations and enhance the care of these patients.
AB - Sarcoidosis is a disease with heterogeneous manifestations and outcomes, varying in part on the basis of organ involvement. Specifically, patients with sarcoidosis at risk for poor outcomes include individuals with treatment-resistant pulmonary sarcoidosis, including fibrotic pulmonary disease and pulmonary hypertension, as well as those with cardiac, neurologic, and multiorgan disease. The limited but available data relating to these patients with high-risk sarcoidosis, defined as those patients with presentations requiring medical intervention to avoid progressive disability or premature death, was evaluated as part of the National Heart, Lung, and Blood Institute's workshop to improve understanding of these disease manifestations. In particular, knowledge gaps that preclude a greater understanding of the pathogenesis and management of these severe sarcoidosis clinical phenotypes were identified in the workshop. Research strategies are proposed to address critical knowledge gaps that would further our understanding of these disease manifestations and enhance the care of these patients.
KW - Cardiac sarcoidosis
KW - Neurosarcoidosis
KW - Pulmonary hypertension
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UR - http://www.scopus.com/inward/citedby.url?scp=85039804400&partnerID=8YFLogxK
U2 - 10.1513/AnnalsATS.201707-566OT
DO - 10.1513/AnnalsATS.201707-566OT
M3 - Article
C2 - 29073361
AN - SCOPUS:85039804400
VL - 14
SP - S437-S444
JO - Annals of the American Thoracic Society
JF - Annals of the American Thoracic Society
SN - 2325-6621
ER -