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Rheumatology

IgG4-related disease

A fibroinflammatory disease of older men that forms tumour-like swellings in the pancreas, bile ducts, salivary and lacrimal glands, retroperitoneum and aorta. Why serum IgG4 neither proves nor excludes it, the biopsy features, the 2019 ACR/EULAR criteria, steroid response, and B-cell depletion (rituximab, inebilizumab in MITIGATE) for relapse.

Tier 2Condition

Written with AI assistance and fact-checked against the sources below. For exam study, not clinical decisions. How notes are made

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At a glance

  • IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that forms tumour-like masses in one or many organs. It is driven by B cells, plasmablasts and CD4+ cytotoxic T cells; IgG4 itself is probably a bystander.
  • Typical patient: man over 50, often with atopy. Onset is subacute; fever and high CRP are unusual.
  • The big risk is mistaking it for cancer (pancreas, bile duct, lymphoma) or missing cancer by calling it IgG4-RD.

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Last updated
14 November 2026
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