Immunoglobulin G4-related disease and idiopathic multicentric Castleman's disease: Confusable immune-mediated disorders

Takanori Sasaki, Mitsuhiro Akiyama, Yuko Kaneko, Tsutomu Takeuchi

Research output: Contribution to journalReview articlepeer-review

12 Citations (Scopus)


IgG4-related disease (IgG4-RD) and idiopathic multicentric Castleman's disease (iMCD) are both rare systemic immune-mediated disorders. However, the pathogenesis differs markedly between the two diseases and differing therapeutic strategies are adopted: IgG4-RD is treated using a moderate dose of glucocorticoids or rituximab, while iMCD therapy involves an IL-6-targeted approach. Nonetheless, some clinical features of IgG4-RD and iMCD overlap, so differential diagnosis is sometimes difficult, even though the classification and diagnostic criteria of the diseases require careful exclusion of the other. The key findings in IgG4-RD are high IgG4:IgG ratio, allergic features and germinal centre expansion involving T follicular helper cells, while iMCD involves polyclonal antibody production (high IgA and IgM levels), sheet-like mature plasma cell proliferation and inflammatory features driven by IL-6. The distribution of organ involvement also provides important clues in both diseases. Particular attention should be given to differential diagnosis using combined clinical and/or pathological findings, because single features cannot distinguish IgG4-RD from iMCD. In the present review, we discuss the similarities and differences between IgG4-RD and iMCD, as well as how to distinguish the two diseases.

Original languageEnglish
Pages (from-to)490-501
Number of pages12
JournalRheumatology (United Kingdom)
Issue number2
Publication statusPublished - 2022 Feb 1


  • Castleman's disease
  • IgG4-related disease
  • biomarkers
  • pathogenesis

ASJC Scopus subject areas

  • Rheumatology
  • Pharmacology (medical)


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