Abstract |
Autoimmune pancreatitis is an unusual form of pancreatitis that is histologically characterised by a massive infiltration of lymphocytes and IgG4-positive plasma cells and storiform fibrosis. The disease is recognised as a pancreatic manifestation of IgG4-related disease. IgG4-related sclerosing cholangitis is a biliary counterpart that is typically associated with autoimmune pancreatitis. Two parallel immunological responses are thought to underlie the pathophysiology of these diseases: a pro-inflammatory, tissue-destructive process and an anti-inflammatory feedback response, which probably relates to IgG4 production. These diseases should be differentiated from conditions with a similar presentation (eg, pancreatobiliary malignancy, primary sclerosing cholangitis) by comparison of serum IgG4 concentration, imaging features, other organ involvement, histology, and steroid responsiveness. Corticosteroids are first-line drugs, although rituximab has been shown to effectively deplete B cells in IgG4-related disease. Although the risk of relapse is high, no standardised treatment protocol exists for relapsed cases.
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Authors | Terumi Kamisawa, Yoh Zen, Takahiro Nakazawa, Kazuichi Okazaki |
Journal | The lancet. Gastroenterology & hepatology
(Lancet Gastroenterol Hepatol)
Vol. 3
Issue 8
Pg. 575-585
(08 2018)
ISSN: 2468-1253 [Electronic] Netherlands |
PMID | 30047448
(Publication Type: Journal Article, Review)
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Copyright | Copyright © 2018 Elsevier Ltd. All rights reserved. |
Chemical References |
- Adrenal Cortex Hormones
- Immunologic Factors
- Rituximab
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Topics |
- Adrenal Cortex Hormones
(therapeutic use)
- Cholangitis, Sclerosing
(drug therapy, epidemiology, immunology, pathology)
- Humans
- Immunoglobulin G4-Related Disease
(drug therapy, epidemiology, immunology, pathology)
- Immunologic Factors
(therapeutic use)
- Incidence
- Pancreatitis
(drug therapy, epidemiology, immunology, pathology)
- Prevalence
- Recurrence
- Remission Induction
- Rituximab
(therapeutic use)
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