ICD-11 code : 4A43.0;
Preferred Label : IgG4 related disease;
ICD-11 definition : IgG4 related syndrome (IgG4-related disease: IgG4-RD) is a clinical disease characterized
by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive
plasma cells. The diagnostic criteria for IgG4 related syndrome have been proposed,
and it may be present in a certain population of patients with a wide variety of diseases,
including Mikulicz disease, autoimmune pancreatitis, hypophysitis, Riedel thyroiditis,
interstitial pneumonitis, interstitial nephritis, prostatitis, lymphadenopathy, retroperitoneal
fibrosis, inflammatory aortic aneurysm, and inflammatory pseudo tumor.;
Origin ID : 99883782;
Automatic exact mappings (from CISMeF team)
- ICD-10 Mapping
IgG4 related syndrome (IgG4-related disease: IgG4-RD) is a clinical disease characterized
by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive
plasma cells. The diagnostic criteria for IgG4 related syndrome have been proposed,
and it may be present in a certain population of patients with a wide variety of diseases,
including Mikulicz disease, autoimmune pancreatitis, hypophysitis, Riedel thyroiditis,
interstitial pneumonitis, interstitial nephritis, prostatitis, lymphadenopathy, retroperitoneal
fibrosis, inflammatory aortic aneurysm, and inflammatory pseudo tumor.
