Back to Search
Start Over
IgG4-related hepatobiliary disease: an overview.
- Source :
-
Nature reviews. Gastroenterology & hepatology [Nat Rev Gastroenterol Hepatol] 2016 Oct; Vol. 13 (10), pp. 601-12. Date of Electronic Publication: 2016 Sep 14. - Publication Year :
- 2016
-
Abstract
- IgG4-related hepatobiliary diseases are part of a multiorgan fibroinflammatory condition termed IgG4-related disease, and include IgG4-related sclerosing cholangitis (IgG4-SC) and IgG4-related hepatopathy. These diseases can present with biliary strictures and/or mass lesions, making them difficult to differentiate from primary sclerosing cholangitis (PSC) or other hepatobiliary malignancies. Diagnosis is based on a combination of clinical, biochemical, radiological and histological findings. However, a gold standard diagnostic test is lacking, warranting the identification of more specific disease markers. Novel assays - such as the serum IgG4:IgG1 ratio and IgG4:IgG RNA ratio (which distinguish IgG4-SC from PSC with high serum IgG4 levels), and plasmablast expansion to recognize IgG4-SC with normal serum IgG4 levels - require further validation. Steroids and other immunosuppressive therapies can lead to clinical and radiological improvement when given in the inflammatory phase of the disease, but evidence for the efficacy of treatment regimens is limited. Progressive fibrosclerotic disease, liver cirrhosis and an increased risk of malignancy are now recognized outcomes. Insights into the genetic and immunological features of the disease have increased over the past decade, with an emphasis on HLAs, T cells, circulating memory B cells and plasmablasts, chemokine-mediated trafficking, as well as the role of the innate immune system.
- Subjects :
- Adrenal Cortex Hormones therapeutic use
Autoimmune Diseases diagnosis
Autoimmune Diseases drug therapy
B-Lymphocytes immunology
Biliary Tract Diseases drug therapy
Biliary Tract Diseases immunology
CD4-Positive T-Lymphocytes immunology
Cholangiopancreatography, Endoscopic Retrograde
Cholangitis, Sclerosing drug therapy
Diagnosis, Differential
Forecasting
Hepatitis, Autoimmune drug therapy
Hepatitis, Autoimmune immunology
Humans
Immunity, Innate
Immunologic Memory immunology
Immunosuppressive Agents therapeutic use
Pancreatitis diagnosis
Pancreatitis drug therapy
Pancreatitis immunology
Paraproteinemias drug therapy
Paraproteinemias immunology
Recurrence
Risk Factors
Terminology as Topic
Treatment Outcome
Biliary Tract Diseases diagnosis
Cholangitis, Sclerosing diagnosis
Hepatitis, Autoimmune diagnosis
Paraproteinemias diagnosis
Subjects
Details
- Language :
- English
- ISSN :
- 1759-5053
- Volume :
- 13
- Issue :
- 10
- Database :
- MEDLINE
- Journal :
- Nature reviews. Gastroenterology & hepatology
- Publication Type :
- Academic Journal
- Accession number :
- 27625195
- Full Text :
- https://doi.org/10.1038/nrgastro.2016.132