Revision 11 for 'IgG4-related disease'

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IgG4 related sclerosing disease

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

Clinical presentation

Clinical manifestations are apparent in the pancreas, biliary tree, gallbladder, salivary gland, retroperitoneum, kidney, lung, 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 can include:

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