Recent Updates on the Relationship between Cancer and Autoimmune Pancreatitis

Intern Med. 2019 Jun 1;58(11):1533-1539. doi: 10.2169/internalmedicine.2210-18. Epub 2019 Feb 1.


Autoimmune pancreatitis (AIP) is now considered a pancreatic manifestation of a newly proposed disease condition, IgG4-related disease (IgG4-RD). IgG4-RD is characterized by enhanced IgG4 antibody responses and multiple organ involvements. Recent epidemiological studies have addressed the incidence of cancer in patients with AIP and/or IgG4-RD. Surprisingly, a significant number of AIP patients were detected with cancer at or within one year of the diagnosis of AIP. Furthermore, around 50% of all cancers detected in AIP patients comprised mainly 3 types (gastric, lung, and prostate cancer). Thus, AIP appears to be associated with cancer of other organs rather than the pancreas itself, which suggests that AIP is not a pre-cancerous condition of the pancreas. Moreover, the simultaneous occurrence of cancer and AIP in many patients has led to the establishment of an attractive concept that AIP might sometimes arise from co-existing cancers as a paraneoplastic syndrome.

Keywords: IgG4-related disease; autoimmune pancreatitis; cancer.

Publication types

  • Review

MeSH terms

  • Acute Disease
  • Autoimmune Diseases / epidemiology
  • Autoimmune Diseases / etiology*
  • Humans
  • Immunoglobulin G4-Related Disease / epidemiology
  • Immunoglobulin G4-Related Disease / etiology*
  • Incidence
  • Neoplasms / complications*
  • Neoplasms / epidemiology
  • Pancreatitis / epidemiology
  • Pancreatitis / etiology*
  • Paraneoplastic Syndromes / epidemiology
  • Paraneoplastic Syndromes / etiology