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Review
. 2014 May 15;5(2):71-81.
doi: 10.4291/wjgp.v5.i2.71.

Review of the diagnosis, classification and management of autoimmune pancreatitis

Derek A O'Reilly  1 , Deep J Malde  1 , Trish Duncan  1 , Madhu Rao  1 , Rafik Filobbos  1
Affiliations
Review

Review of the diagnosis, classification and management of autoimmune pancreatitis

Derek A O'Reilly et al. World J Gastrointest Pathophysiol. .

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: Autoimmune pancreatitis; Autoimmunity; Immunoglobulin G4-related disease; Pancreatic cancer; Pancreatitis.

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Figures

Figure 1
Figure 1
Computed tomography. A: Computed tomography (CT) findings in autoimmune pancreatitis: Showing diffuse enlargement and a “sausage like” appearance of the pancreas (arrow); B: Axial contrast enhanced CT image demonstrating a characteristic low signal rim or halo surrounding the body and tail of the pancreas in another patient with autoimmune pancreatitis.
Figure 2
Figure 2
Focal enlargement of the pancreatic parenchyma in the head of the pancreas (arrow), and dilatation of the intrahepatic bile ducts visible (arrowheads).
Figure 3
Figure 3
Endoscopic retrograde cholangiopancreatography findings of multiple and focal strictures and dilatation in the intrahepatic bile ducts in autoimmune pancreatitis.
Figure 4
Figure 4
A strategy for distinguishing autoimmune pancreatitis from pancreatic cancer (based upon the Mayo clinic strategy[23]). CT: Computed tomography; MRI: Magnetic resonance imaging; AIP: Autoimmune pancreatitis.
Figure 5
Figure 5
Axial computed tomography image. A: Demonstrating a characteristic sausage shaped enlarged pancreas with surrounding halo in keeping with autoimmune pancreatitis; B: From the same patient 8 mo later following corticosteroid therapy demonstrating response to treatment.
See this image and copyright information in PMC

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