Revision 22 for 'IgG4-related disease'

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IgG4-related disease

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

Terminology

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.

Epidemiology

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

Clinical presentation

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.

Pathology

The disease spectrum can include:

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