Defining Relapse and Disease Activity in Neurosarcoidosis: A Systematic Review and Proposed Framework

Area of Science:

• Neurology
• Immunology
• Systematic Reviews

Background:

• Central nervous system (CNS) neurosarcoidosis lacks standardized disease activity characterization.
• Existing literature exhibits significant heterogeneity in monitoring metrics and definitions.
• Current approaches often focus on single domains or analyze multiple domains independently.

Purpose of the Study:

• To systematically review longitudinal monitoring metrics for CNS neurosarcoidosis.
• To establish an evidence-based clinical framework for characterizing, monitoring, and communicating disease activity.
• To develop a standardized approach applicable to both clinical practice and research.

Main Methods:

• Conducted a systematic review of articles reporting longitudinal monitoring or outcome metrics for neurosarcoidosis.
• Included studies with at least one relevant monitoring metric.
• Developed a multidomain framework integrating clinical, imaging, and laboratory data.

Main Results:

• Identified 67 relevant studies from 387 initially screened.
• Found most studies lacked formal definitions and focused on isolated domains.
• The proposed framework integrates clinical, imaging, and laboratory domains for comprehensive assessment.
• The framework categorizes disease status (improved, stable, worsened, resolved) within each domain.

Conclusions:

• A systematic review revealed significant heterogeneity in CNS neurosarcoidosis disease activity assessment.
• The proposed multidomain framework offers a standardized approach to characterize disease activity.
• Further validation of this framework can enhance research standardization and clinical communication.