IgG4-related disease

Dr Henry Knipe and Dr Yuranga Weerakkody et al.

IgG4-related disease (IgG4-RD) is a systemic disease that is characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration of various organs.

This condition has been known by many other names in the past, such as IgG4-related sclerosing disease, IgG4-related systemic disease, IgG4-related systemic sclerosing disease, IgG4-syndrome, and IgG4-associated disease, however a consensus in 2012 agreed upon the use of IgG4-related disease.

IgG4-related diseases are more common in middle age or elderly males.

Clinical manifestations are apparent in the pancreas, biliary tree, gallbladder, salivary gland, retroperitoneum, kidney, lung, pituitary gland, and prostate, in which tissue fibrosis with obliterative phlebitis is pathologically induced. These organ systems may be either synchronously or sequentially involved.

The disease spectrum may include:

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Article information

rID: 17290
Section: Gamuts
Synonyms or Alternate Spellings:
  • IgG4-related disease (IgG4-RD)
  • IgG4-related sclerosing disease
  • IgG4-related systemic disease
  • IgG4-related systemic sclerosing disease
  • IgG4-syndrome
  • IgG4-associated disease

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Cases and figures

  • Post-contrast T1-...
    Case 1: IgG4-related hypophysitis
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  • Case 2: IgG4-related AIP
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  • Case 3: IgG4-related AIP
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