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- Title
Immunoglobulin G4–related disease and idiopathic multicentric Castleman's disease: confusable immune-mediated disorders.
- Authors
Sasaki, Takanori; Akiyama, Mitsuhiro; Kaneko, Yuko; Takeuchi, Tsutomu
- Abstract
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.
- Subjects
THERAPEUTIC use of glucocorticoids; RITUXIMAB; IMMUNOGLOBULINS; CASTLEMAN'S disease; AUTOIMMUNE diseases; DIFFERENTIAL diagnosis; CELL proliferation
- Publication
Rheumatology, 2022, Vol 61, Issue 2, p490
- ISSN
1462-0324
- Publication type
Article
- DOI
10.1093/rheumatology/keab634