IgG4-Related Disease: The Great Pretender

[Featured image of solid pseudopapillary tumor (SPT) of the pancreas.]

By Martina M. McGrath, MD
March 24, 2017

Retroperitoneal fibrosis, autoimmune pancreatitis, Reidel’s thyroiditis, sclerosing cholangitis and Mikulicz’s disease, sclerosing aortitis and periaortitis – what do all of these conditions have in common?

IgG4-related disease (IgG4-RD) is a chronic, fibro-inflammatory condition with a characteristic histological appearance including dense lymphoplasmacytic infiltration with large numbers of IgG4-positive plasma cells, storiform fibrosis, obliterative phlebitis,and eosinophil infiltration. 1 It is now recognized as the cause of a large number of chronic inflammatory conditions, previously considered idiopathic, including those listed above. IgG4-RD is the most common cause of Type I autoimmune pancreatitis. 2 Continue reading “IgG4-Related Disease: The Great Pretender”