Clinical spectrum and management

Einar S. Björnsson, Keith Lindor

Research output: Chapter in Book/Report/Conference proceedingChapter


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 languageEnglish (US)
Title of host publicationAutoimmune (IgG4-Related) Pancreatitis and Cholangitis
PublisherSpringer New York
Number of pages9
ISBN (Print)9781441964304, 1441964290, 9781441964298
StatePublished - Feb 1 2014
Externally publishedYes

ASJC Scopus subject areas

  • Medicine(all)

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  • Cite this

    Björnsson, E. S., & Lindor, K. (2014). Clinical spectrum and management. In Autoimmune (IgG4-Related) Pancreatitis and Cholangitis (pp. 171-179). Springer New York.