IgG4-related disease (IgG4-RD) is a relatively newly described disease entity. Autoimmune pancreatitis is its most widely recognized and best characterized manifestation. However, extrapancreatic lesions are common, such as cholangitis and retroperitoneal fibrosis. The term IgG<inf>4</inf>-related sclerosing cholangitis (IgG<inf>4</inf>-SC) biliary manifestation of IgG4-RD. Biliary strictures diagnosed and treated with steroids early in the course of IgG4-SC can resolve after successful therapy. This feature defines IgG<inf>4</inf>-SC which is histologically characterized by infiltration by IgG4-positive plasma cells. Though biliary strictures have been found to resolve with steroids, relapse is common. The long-term outcome is unclear. According to current guidelines, serum IgG4 levels should be measured in all patients with possible primary sclerosing cholangitis (PSC), to rule out IgG<inf>4</inf>-SC. PSC patients with elevated IgG4 levels seem to have a worse prognosis than those with normal IgG4 levels.
|Original language||English (US)|
|Title of host publication||Autoimmune (IgG4-Related) Pancreatitis and Cholangitis|
|Publisher||Springer New York|
|Number of pages||9|
|ISBN (Print)||9781441964304, 1441964290, 9781441964298|
|State||Published - Feb 1 2014|
ASJC Scopus subject areas