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Published on: July 30, 2011
Systemic extrapancreatic lesions associated with autoimmune pancreatitis
Hirotaka Ohara1, Takahiro Nakazawa, Tomoaki Ando
1Department of Internal Medicine and Bioregulation, Nagoya City University Graduate School of Medical Sciences, 1 Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya, Aichi, 467-8601, Japan.
Journal of Gastroenterology
|May 24, 2007
Summary
Autoimmune pancreatitis (AIP) and sclerosing cholangitis (SC) are linked, but distinct from primary sclerosing cholangitis (PSC). AIP-SC responds to steroids, unlike PSC, and shows specific cholangiographic and IgG4+ cell differences.
Area of Science:
- Gastroenterology and Immunology
Background:
- Autoimmune pancreatitis (AIP) frequently co-occurs with sclerosing cholangitis (SC).
- Cholangiographic findings in SC with AIP can mimic primary sclerosing cholangitis (PSC), leading to diagnostic challenges.
- Distinguishing between SC with AIP and PSC is crucial as only SC with AIP responds to corticosteroid therapy.
Purpose of the Study:
- To differentiate SC with AIP from PSC using cholangiographic, clinical, and immunohistochemical findings.
- To investigate the association of AIP with systemic extrapancreatic lesions.
- To explore the role of IgG4 in AIP and its potential autoimmune targets.
Main Methods:
- Comparative analysis of cholangiographic features in SC with AIP and PSC.
- Evaluation of clinical data, including laboratory markers like IgG4 levels.
- Immunohistochemical examination for IgG4-positive plasma cell infiltration in affected tissues.
- Serological testing for IgG4 autoantibodies in AIP patients.
Main Results:
- Distinct cholangiographic patterns differentiate SC with AIP (segmental strictures, lower CBD strictures) from PSC (band-like strictures, beaded appearance).
- Higher gamma-globulin, IgG, and IgG4 levels were observed in AIP patients with systemic extrapancreatic lesions.
- Marked IgG4+ plasma cell infiltration was prevalent in AIP tissues, contrasting with lower infiltration in PSC.
- AIP sera showed reactivity with normal epithelia, suggesting potential IgG4 autoantibodies targeting various organs.
Conclusions:
- Cholangiography, clinical data, and IgG4 staining effectively distinguish SC with AIP from PSC.
- AIP is associated with systemic extrapancreatic manifestations and elevated IgG4 levels.
- AIP may involve IgG4 autoantibodies targeting multiple organs, indicating a distinct autoimmune disease process.
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