High-Risk Sarcoidosis. Current Concepts and Research Imperatives

Area of Science:

• Pulmonary Medicine
• Rheumatology
• Immunology

Background:

• Sarcoidosis presents with diverse manifestations and outcomes influenced by organ involvement.
• Certain sarcoidosis patients face poor prognoses, including those with treatment-resistant pulmonary disease (fibrotic lung disease, pulmonary hypertension), cardiac, neurologic, or multiorgan involvement.
• High-risk sarcoidosis necessitates medical intervention to prevent disability or premature death.

Purpose of the Study:

• To evaluate existing data on high-risk sarcoidosis phenotypes.
• To identify critical knowledge gaps in understanding the pathogenesis and management of severe sarcoidosis.
• To propose research strategies for advancing the care of patients with severe sarcoidosis.

Main Methods:

• A workshop convened by the National Heart, Lung, and Blood Institute (NHLBI) was utilized.
• Evaluation of limited available data on high-risk sarcoidosis patients was performed.
• Identification of knowledge gaps hindering understanding of severe sarcoidosis clinical phenotypes.

Main Results:

• Heterogeneity in sarcoidosis outcomes is linked to specific organ involvement.
• Patients with fibrotic pulmonary sarcoidosis, pulmonary hypertension, cardiac, neurologic, or multiorgan disease are at higher risk.
• Significant knowledge gaps exist regarding the pathogenesis and optimal management of severe sarcoidosis.

Conclusions:

• Further research is crucial for understanding severe sarcoidosis.
• Addressing identified knowledge gaps will enhance the care of patients with high-risk sarcoidosis.
• Proposed research strategies aim to improve outcomes for individuals with severe sarcoidosis manifestations.