TY - JOUR
T1 - Clinical presentation and treatment of high-risk sarcoidosis
AU - Perlman, David M.
AU - Sudheendra, Muthya Tejasvini
AU - Furuya, Yuka
AU - Shenoy, Chetan
AU - Kalra, Rajat
AU - Roukoz, Henri
AU - Markowitz, Jeremy
AU - Maier, Lisa A.
AU - Bhargava, Maneesh
N1 - Publisher Copyright:
© 2021 by the American Thoracic Society.
PY - 2021/12
Y1 - 2021/12
N2 - Sarcoidosis is a multisystem disease of unknown cause with heterogeneous clinical manifestations and variable course. Spontaneous remissions occur in some patients, whereas others have progressive disease impacting survival, organ function, and quality of life. Four high-risk sarcoidosis phenotypes associated with chronic inflammation have recently been identified as highpriority areas for research. These include treatment-refractory pulmonary disease, cardiac sarcoidosis, neurosarcoidosis, and multiorgan sarcoidosis. Significant gaps currently exist in the understanding of these high-risk manifestations of sarcoidosis, including their natural history, diagnostic criteria, biomarkers, and the treatment strategy, such as the ideal agent, optimal dose, and treatment duration. The use of registries with wellphenotyped patients is a critical first step to study high-risk sarcoidosis manifestations systematically. We review the diagnostic and treatment approach to high-risk sarcoidosis manifestations. Appropriately identifying these disease subgroups will help enroll well-phenotyped patients in sarcoidosis registries and clinical trials, a necessary step to narrow existing gaps in understanding of this enigmatic disease.
AB - Sarcoidosis is a multisystem disease of unknown cause with heterogeneous clinical manifestations and variable course. Spontaneous remissions occur in some patients, whereas others have progressive disease impacting survival, organ function, and quality of life. Four high-risk sarcoidosis phenotypes associated with chronic inflammation have recently been identified as highpriority areas for research. These include treatment-refractory pulmonary disease, cardiac sarcoidosis, neurosarcoidosis, and multiorgan sarcoidosis. Significant gaps currently exist in the understanding of these high-risk manifestations of sarcoidosis, including their natural history, diagnostic criteria, biomarkers, and the treatment strategy, such as the ideal agent, optimal dose, and treatment duration. The use of registries with wellphenotyped patients is a critical first step to study high-risk sarcoidosis manifestations systematically. We review the diagnostic and treatment approach to high-risk sarcoidosis manifestations. Appropriately identifying these disease subgroups will help enroll well-phenotyped patients in sarcoidosis registries and clinical trials, a necessary step to narrow existing gaps in understanding of this enigmatic disease.
KW - Cardiac sarcoidosis
KW - High-risk sarcoidosis
KW - Multiorgan sarcoidosis
KW - Neurosarcoidosis
KW - Progressive pulmonary sarcoidosis
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U2 - 10.1513/AnnalsATS.202102-212CME
DO - 10.1513/AnnalsATS.202102-212CME
M3 - Review article
C2 - 34524933
AN - SCOPUS:85120634789
SN - 2329-6933
VL - 18
SP - 1935
EP - 1947
JO - Annals of the American Thoracic Society
JF - Annals of the American Thoracic Society
IS - 12
ER -