Review Article

From Pathogenesis, Clinical Manifestation, and Diagnosis to Treatment: An Overview on Autoimmune Pancreatitis

Table 1

Comparisons of the two types of AIP.

CharacteristicsType 1Type 2

Other nomenclatures [5]LPSPIDCP
AIP without GELAIP with GEL
IgG4 relatedIgG4 unrelated

Ethnic [5]Asia > United States, EuropeEurope > United States > Asian

Age [2, 5, 18]60 years or olderA decade younger

Sex [5]Usually maleEqual

Symptom [5]Obstructive jaundice oftenObstructive jaundice often
Abdominal pain rareAbdominal pain common
Pancreas swelling commonPancreas swelling common

Serology [2, 5]High serum IgG4, auto-Ab+Normal serum IgG4, auto-Ab−

Histopathology [5]Lymphocyte and plasmacyte infiltration and fibrosisGranulocyte epithelial lesion often with destruction and obliteration of the pancreatic duct
Infiltration of IgG4 plasma cells

Extrapancreatic lesion [5, 15, 17]Sclerosing cholangitis Unrelated with OOI
Sclerosing sialadenitis
Retroperitoneal fibrosis, etc.

Ulcerative colitis [2, 5]RareOften

Histology needed for diagnosis [5]NoYes

Respond to steroid [2, 5]ResponsiveResponsive

Relapse rate [5]HighLow