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World J Gastrointest Pathophysiol. May 15, 2014; 5(2): 71-81
Published online May 15, 2014. doi: 10.4291/wjgp.v5.i2.71
Review of the diagnosis, classification and management of autoimmune pancreatitis
Derek A O’Reilly, Deep J Malde, Trish Duncan, Madhu Rao, Rafik Filobbos
Derek A O’Reilly, Deep J Malde, Trish Duncan, Department of Hepatobiliary and Pancreatic Surgery, North Manchester General Hospital, Manchester M8 5RB, United Kingdom
Derek A O’Reilly, Institute of Cancer Sciences, University of Manchester, Manchester Academic Health Science Centre, Manchester M8 5RB, United Kingdom
Madhu Rao, Department of Histopathology, North Manchester General Hospital, Manchester M8 5RB, United Kingdom
Rafik Filobbos, Department of Radiology, North Manchester General Hospital, Manchester M8 5RB, United Kingdom
Author contributions: O’Reilly DA, Malde DJ and Duncan T wrote the clinical sections; Rao M contributed to the pathological section; Filobbos R supplied the radiology section and images.
Correspondence to: Derek A O’Reilly, PhD, FRCS, Consultant HPB Surgeon and Honorary Senior Lecturer, Department of Hepatobiliary and Pancreatic Surgery, North Manchester General Hospital, Manchester M8 5RB,United Kingdom. doreilly@doctors.org.uk
Telephone: +44-161-7202277 Fax: +44-161-7202228
Received: December 31, 2013
Revised: March 27, 2014
Accepted: April 17, 2014
Published online: May 15, 2014
Abstract

Autoimmune pancreatitis (AIP) is a rare form of chronic pancreatitis, with as yet undetermined incidence and prevalence in the general population. Our understanding of it continues to evolve. In the last few years, 2 separate subtypes have been identified: type 1 AIP has been recognised as the pancreatic manifestation of a multiorgan disease, named immunoglobulin G4 (IgG4)-related disease while type 2 AIP is a pancreas specific disorder not associated with IgG4. International criteria for the diagnosis of AIP have been defined: the HISORt criteria from the Mayo clinic, the Japan consensus criteria and, most recently, the international association of pancreatology “International Consensus Diagnostic Criteria”. Despite this, in clinical practice it can still be very difficult to confirm the diagnosis and differentiate AIP from a pancreatic cancer. There are no large studies into the long-term prognosis and management of relapses of AIP, and there is even less information at present regarding the Type 2 AIP subtype. Further studies are necessary to clarify the pathogenesis, treatment and long-term outcomes of this disease. Critically for clinicians, making the correct diagnosis and differentiating the disease from pancreatic cancer is of the utmost importance and the greatest challenge.

Keywords: Pancreatitis, Autoimmunity, Pancreatic cancer, Autoimmune pancreatitis, Immunoglobulin G4-related disease

Core tip: Type 1 autoimmune pancreatitis (AIP) is the pancreatic manifestation of a multiorgan disease, named immunoglobulin G4 (IgG4)-related disease while type 2 AIP is a pancreas specific disorder not associated with IgG4. Making the correct diagnosis and differentiating the disease from pancreatic cancer is of the utmost importance; an agreed diagnostic pathway should be in place and a multidisciplinary approach taken with each patient.