Current Concepts and Diagnosis of IgG4-Related Pancreatitis (Type 1 AIP)

Semin Liver Dis. 2016 Aug;36(3):257-73. doi: 10.1055/s-0036-1584318. Epub 2016 Jul 28.


Although now considered to be a member of the systemic entity of immunoglobulin G4- (IgG4-) related disease, IgG4-related pancreatitis is generally referred to as type 1 autoimmune pancreatitis (AIP). Type 1 AIP was established based on a pathological background of lymphoplasmacytic sclerosing pancreatitis, high serum IgG4 concentration, and abundant IgG4-bearing plasma cell infiltration. The characteristic clinical features of type 1 AIP, such as elderly male preponderance, obstructive jaundice, and mass-forming lesions in the pancreas, often mimic those of pancreatic cancer. However, because AIP responds favorably to corticosteroid treatment, careful differentiation from pancreatic cancer is required. An AIP diagnosis is currently based on the 2011 International Consensus Diagnostic Criteria for AIP, which are based on high sensitivity, selectivity, and accuracy. Over the long term, AIP can progress to a chronic condition, with pancreatic stone formation and atrophy resembling that of chronic pancreatitis. Although AIP has been linked to the complication of malignancies, it remains controversial whether an association exists between the disease and tumor formation.

Publication types

  • Review
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Autoimmune Diseases / diagnosis*
  • Autoimmune Diseases / genetics
  • Autoimmune Diseases / physiopathology
  • Biomarkers / blood
  • Cholangiopancreatography, Endoscopic Retrograde
  • Diagnosis, Differential
  • Disease Progression
  • Humans
  • Immunoglobulin G / blood*
  • Immunoglobulin G / immunology
  • Jaundice, Obstructive / etiology
  • Male
  • Pancreatic Ducts / diagnostic imaging
  • Pancreatic Ducts / pathology*
  • Pancreatitis / diagnosis*
  • Pancreatitis / genetics
  • Pancreatitis / physiopathology
  • Positron-Emission Tomography
  • Recurrence
  • Tomography, X-Ray Computed
  • Ultrasonography


  • Adrenal Cortex Hormones
  • Biomarkers
  • Immunoglobulin G