Autoimmune pancreatitis: proposal of IgG4-related sclerosing disease

Autoimmune pancreatitis (AIP) is a peculiar type of pancreatitis of presumed autoimmune etiology. Many new clinical aspects of AIP have been clarified during the past 10 years, and AIP has become a distinct entity recognized worldwide. However, its precise pathogenesis or pathophysiology remains unc...

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Bibliographic Details
Published in:Journal of gastroenterology 2006-06, Vol.41 (7), p.613-625
Main Authors: Kamisawa, Terumi, Okamoto, Atsutake
Format: Article
Language:English
Subjects:
Autoimmune Diseases - classification
Autoimmune Diseases - immunology
Autoimmune Diseases - physiopathology
Cholangitis, Sclerosing - immunology
Cholangitis, Sclerosing - physiopathology
Humans
Immunoglobulin G - blood
Pancreatitis - classification
Pancreatitis - immunology
Pancreatitis - physiopathology
Plasma Cells
Retroperitoneal Fibrosis - immunology
Retroperitoneal Fibrosis - physiopathology
Review
Sclerosis - classification
Sclerosis - immunology
Sclerosis - physiopathology
Terminology as Topic
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Summary:Autoimmune pancreatitis (AIP) is a peculiar type of pancreatitis of presumed autoimmune etiology. Many new clinical aspects of AIP have been clarified during the past 10 years, and AIP has become a distinct entity recognized worldwide. However, its precise pathogenesis or pathophysiology remains unclear. As AIP dramatically responds to steroid therapy, accurate diagnosis of AIP is necessary to avoid unnecessary surgery. Characteristic dense lymphoplasmacytic infiltration and fibrosis in the pancreas may prove to be the gold standard for diagnosis of AIP. However, since it is difficult to obtain sufficient pancreatic tissue, AIP should be diagnosed currently based on the characteristic radiological findings (irregular narrowing of the main pancreatic duct and enlargement of the pancreas) in combination with serological findings (elevation of serum gamma-globulin, IgG, or IgG4, along with the presence of autoantibodies), clinical findings (elderly male preponderance, fluctuating obstructive jaundice without pain, occasional extrapancreatic lesions, and favorable response to steroid therapy), and histopathological findings (dense infiltration of IgG4-positive plasma cells and T lymphocytes with fibrosis and obliterative phlebitis in various organs). It is apparent that elevation of serum IgG4 levels and infiltration of abundant IgG4-positive plasma cells into various organs are rather specific to AIP patients. We propose a new clinicopathological entity, "IgG4-related sclerosing disease", and suggest that AIP is a pancreatic lesion reflecting this systemic disease.
ISSN:0944-1174
1435-5922
DOI:10.1007/s00535-006-1862-6