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Gastroenterol Clin North Am. 2017 Jun;46(2):195-216. doi: 10.1016/j.gtc.2017.01.001.

IgG4-related Disease and the Liver.

Author information

1
Department of Pathology, Massachusetts General Hospital, Harvard Medical School, 55 Fruit Street, Boston, MA 01224, USA.
2
Department of Pathology, Massachusetts General Hospital, Harvard Medical School, 55 Fruit Street, Boston, MA 01224, USA. Electronic address: vikramdirdeshpande@gmail.com.

Abstract

Pathologists are likely to encounter IgG4-related disease in several organ systems. This article focuses on helping pathologists diagnose IgG4-related disease in the hepatobiliary system. Missing the diagnosis can result in unnecessary organ damage and/or unnecessary surgical and cancer therapy. In the liver, tumefactive lesion(s) involving the bile ducts with storiform fibrosis and an IgG4-enriched lymphoplasmacytic infiltrate are highly concerning for IgG4-related disease. The recent identification of oligoclonal populations of T cells and B cells in IgG4-related disease may lead to molecular tests, new therapeutics, and a greater mechanistic understanding of the disease.

KEYWORDS:

Autoimmune pancreatitis; IgG4; IgG4-related disease; IgG4-related sclerosing cholangitis; Primary sclerosing cholangitis

PMID:
28506361
DOI:
10.1016/j.gtc.2017.01.001
[Indexed for MEDLINE]

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