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World J Gastroenterol. Dec 28, 2022; 28(48): 6867-6874
Published online Dec 28, 2022. doi: 10.3748/wjg.v28.i48.6867
Table 1 Characteristics of the two subtypes of autoimmune pancreatitis
Characteristic
AIP-1
AIP-2
Male/female ratio3/11/1
Mean age65 yr40 yr
Geographical distributionAsia > Europe and United StatesEurope and United States > Asia
Clinical presentationJaundice 60%-80%. Acute pancreatitis 15%. Weight loss 65%Acute pancreatitis 80%. Jaundice < 30%
Biological presentationIgG4 > 1.35 g/L (sensitivity 70%, specificity 93%). IgG4 > 2.7 g/L (sensitivity 53%, specificity 99%). Lipase < 3xN. Cholestasis: > 80% of cases. Diabetes: 65% of cases. Insulin-dependent diabetes: 20% of cases. Exocrine pancreatic insufficiency: 40% of casesUnspecific. Lipase > 3xN. Rare endocrine and exocrine pancreatic insufficiency
Histological criteriaLymphoplasmacytic infiltration without neutrophils. Storiform fibrosis. Obliterative venulitis. IgG4 plasma cells > 10 in a high-power fieldDestruction of inter- and intralobular ducts by neutrophils (granulocytic epithelial lesions). Few or no IgG4 plasma cells
Relapse rate after corticosteroid therapy> 30%< 15%