IgG4-Related Disease

IgG4-Related Disease is indexed under Autoimmune & Inflammatory, Immune-Mediated Fibroinflammatory. Also known as IgG4-RD.

US prevalence

~0.28-1 per 100,000 per year (likely underestimated) Source: ACR; IgG4-RD Foundation. This figure comes from a professional society, registry, or study rather than a federal source. Label: REPORTED.

ICD-10-CM codes

D89.811, D89.812, D89.813

Typical onset

Typically >50 years; median ~60

Course

managed

Body systems

pancreatic, hepatobiliary, salivary, renal, retroperitoneal

Sex differences

M:F ~2-3:1 for pancreatic/retroperitoneal; closer to equal for salivary gland

Racial and ethnic differences

First described in Japan; now recognized worldwide

What this page does not establish

These figures describe populations, not individuals, and none of them is a diagnosis or a treatment recommendation. Prevalence estimates are approximate and reflect published ranges. Disparity figures are reported patterns, not causes.

Taxonomy last reviewed 2026-05-19. Each figure on the interactive page carries its own source and retrieval date, which can be later than this review.

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