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February 25, 2026JCR Journal of Clinical Rheumatology0 citations

A Systematic Review of Erdheim-Chester Disease and IgG4-Related Disease

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SGSimhachalam GurugubelliRKRithik Naik KorraVMVenkata Sai Abhilash Meda

Key Points

  • The aim is to differentiate Erdheim-Chester Disease from IgG4-Related Disease to enhance diagnosis and treatment.
  • Conducted a systematic review of existing literature
  • Integrated findings from genetic testing and imaging studies
  • Analyzed neurological features and treatment responses
  • Improved diagnostic accuracy through a structured approach
  • Reduction in misdiagnoses reported
  • Enhanced treatment targeting for better patient outcomes

Abstract

This review provides a structured, multimodal approach to distinguish ECD from IgG4-RD, which improves diagnostic accuracy by integrating genetic testing, advanced imaging, neurological features, and treatment response. This allows clinicians to avoid misdiagnoses, implement targeted treatments, and improve patient outcomes.

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Cite This Study

Gurugubelli et al. (2025) studied this question.

synapsesocial.com/papers/699e927bf5123be5ed050347https://doi.org/10.1097/rhu.0000000000002299
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