O’Reilly DA, Malde DJ, Duncan T, Rao M, Filobbos R. Review of the diagnosis, classification and management of autoimmune pancreatitis. World J Gastrointest Pathophysiol 2014; 5(2): 71-81 [PMID: 24891978 DOI: 10.4291/wjgp.v5.i2.71]
Corresponding Author of This Article
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
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Topic Highlight
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Typical; diffusely enlarged gland with diffuse rim enhancement, diffusely irregular attenuated pancreatic duct
Other; focal pancreatic mass or enlargement; focal pancreatic duct stricture; pancreatic duct stricture, pancreatic atrophy; pancreatic calcification or pancreatitis
Serology
Elevated serum IgG4 level
Other organ involvement
Hilar/intrahepatic biliary strictures, persistent distal biliary strictures, parotid or lacrimal gland involvement, mediastinal lymphadenopathy or retroperitoneal fibrosis
Response to steroid therapy
Resolution/Marked improvement of pancreatic or extrapancreatic manifestion with steroid therapy
Table 2 International consensus diagnostic criteria for type 1 autoimmune pancreatitis
Diagnosis of type 1 AIP
Diagnosis
Cardinal feature
Imaging evidence
Collateral evidence
Definitive type 1
Histology
Typical/inderminate
Confirmed LPSP
Imaging
Typical Inderminate
Any level 1/2 ≥ 2 level 1
Steroid response
Indeterminate
Level 1 S/OOI and Rt OR Level 1 D and level 2 S/OOI/H and Rt
Probable type 1
Indeterminate
Level 2 S/OOI/H and Rt
Table 3 International consensus diagnostic criteria for type 2 autoimmune pancreatitis
Diagnosis of type 2 AIP
Diagnosis
Imaging evidence
Collateral evidence
Definitive type 2
Typical/indeterminate
Histologically confirmed or clinical inflammatory bowel disease and level 2H and Rt
Probable type 2
Typical/indeterminate
Level 2 H/clinical inflammatory bowel disease and Rt
Table 4 International consensus diagnostic criteria level 1 and 2 criteria for type 1 and 2 autoimmune pancreatitis
Type 1 AIP
Criterion
Level 1
Level 2
Parenchymal imaging
Typical: Diffuse enlargement with delayed enhancement
Indeterminate: Focal enlargement with delayed enhancement
Ductal imaging (ERP)
Long or multiple strictures (> 1/3 duct length) without upstream dilatation
Focal narrowing without upstream dilatation (< 5 mm)
Serology
IgG4 > 2x upper limit
IgG4 1-2x upper limit
Other organ involvement
Extrapancreatic organ histology. Any 3 of : 1 Lymphoplasmacytic infiltration with fibrosis and without granulocytic infiltration 2 Storiform fibrosis 3 Obliterative phlebitis 4 > 10 cells/HPF IgG4-positive cells Typical radiology. Any one of: 1 Segmental/multiple proximal or distal biliary stricture 2 Retroperitoneal fibrosis
Extrapancreatic organ histology including bile duct biopsies. Both of: 1 Marked lymphoplasmacytic infiltration without granulocytic infiltration 2 10 cells/HPF IgG4-positive cells Physical or radiological evidence of at least one of: 1 Enlarged salivary/lachrymal glands 2 Renal involvement
Rapid (< 2 wk) radiological demonstration of marked improvement in pancreatic/extrapancreatic manifestations
Type 2 AIP
Parenchymal imaging
Typical: Diffuse enlargement with delayed enhancement
Indeterminate: Focal enlargement with delayed enhancement
Ductal Imaging (ERCP)
Long (> 1/3 duct length) or multiple strictures without upstream dilatation
Focal narrowing without marked upstream dilatation (< 5 mm)
Other organ involvement
Clinically diagnosed inflammatory bowel disease
Histology of pancreas
IDCP. Both of: 1 Granulocytic infiltration of duct wall with or without acinar inflammation 2 0-10 cells/HPF IgG4-positive cells
Both of : 1 Granulocytic and lymphoplasmacytic acinar infiltrate 2 0-10 cells/HPF IgG4-positive cells
Response to steroid (Rt)
Rapid (< 2 wk) radiological demonstration of marked improvement in manifestations
Citation: O’Reilly DA, Malde DJ, Duncan T, Rao M, Filobbos R. Review of the diagnosis, classification and management of autoimmune pancreatitis. World J Gastrointest Pathophysiol 2014; 5(2): 71-81