Clinical Presentation and Treatment of High-Risk Sarcoidosis

Ann Am Thorac Soc. 2021 Dec;18(12):1935-1947. doi: 10.1513/AnnalsATS.202102-212CME.


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 high-priority 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 well-phenotyped 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.

Keywords: cardiac sarcoidosis; high-risk sarcoidosis; multiorgan sarcoidosis; neurosarcoidosis; progressive pulmonary sarcoidosis.

Publication types

  • Review

MeSH terms

  • Central Nervous System Diseases*
  • Humans
  • Lung
  • Phenotype
  • Quality of Life
  • Sarcoidosis* / diagnosis
  • Sarcoidosis* / therapy