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01.12.2014 | Review | Ausgabe 1/2014 Open Access

Orphanet Journal of Rare Diseases 1/2014

Autoimmune pancreatitis can develop into chronic pancreatitis

Orphanet Journal of Rare Diseases > Ausgabe 1/2014
Masahiro Maruyama, Takayuki Watanabe, Keita Kanai, Takaya Oguchi, Jumpei Asano, Tetsuya Ito, Yayoi Ozaki, Takashi Muraki, Hideaki Hamano, Norikazu Arakura, Shigeyuki Kawa
Wichtige Hinweise

Electronic supplementary material

The online version of this article (doi:10.​1186/​1750-1172-9-77) contains supplementary material, which is available to authorized users.

Competing interests

The authors declare that they have no competing interests.

Authors’ contributions

MM and SK conceived the project and wrote the manuscript. All authors were involved in recruitment of patients and diagnosis of AIP, actively exchanged ideas, and approved the final version.


Autoimmune pancreatitis (AIP) has been recognized as a distinct type of pancreatitis that is possibly caused by autoimmune mechanisms. AIP is characterized by high serum IgG4 and IgG4-positive plasma cell infiltration in affected pancreatic tissue. Acute phase AIP responds favorably to corticosteroid therapy and results in the amelioration of clinical findings. However, the long-term prognosis and outcome of AIP remain unclear. We have proposed a working hypothesis that AIP can develop into ordinary chronic pancreatitis resembling alcoholic pancreatitis over a long-term course based on several clinical findings, most notably frequent pancreatic stone formation. In this review article, we describe a series of study results to confirm our hypothesis and clarify that: 1) pancreatic calcification in AIP is closely associated with disease recurrence; 2) advanced stage AIP might have earlier been included in ordinary chronic pancreatitis; 3) approximately 40% of AIP patients experience pancreatic stone formation over a long-term course, for which a primary risk factor is narrowing of both Wirsung’s and Santorini’s ducts; and 4) nearly 20% of AIP patients progress to confirmed chronic pancreatitis according to the revised Japanese Clinical Diagnostic Criteria, with independent risk factors being pancreatic head swelling and non-narrowing of the pancreatic body duct.
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