High-Risk Sarcoidosis. Current Concepts and Research Imperatives

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.