Autoimmune pancreatitis, also called AIP, is a chronic inflammation that is thought to be caused by the body's immune system attacking the pancreas and that responds to steroid therapy. Two subtypes of AIP are now recognized, type 1 and type 2.
Type 1 AIP is also called IgG4-related pancreatitis and is part of a disease called IgG4-related disease (IgG4-RD) that often affects multiple organs including the pancreas, bile ducts in the liver, salivary glands, kidneys and lymph nodes.
Type 2 AIP seems to affect only the pancreas, although about one-third of people with type 2 AIP have associated inflammatory bowel disease.
Both subtypes of autoimmune pancreatitis are treated with steroids, which in many people dramatically improve the condition.
Autoimmune pancreatitis is a rare, newly recognized disease and can be mistakenly diagnosed as pancreatic cancer. Both conditions have similar signs and symptoms, but very different treatments, so it is very important to distinguish one from another.
Dec. 20, 2014
- Hart PA, et al. Long-term outcomes of autoimmune pancreatitis: A multicentre, international analysis. Gut. 2013;62:1771.
- Chari ST, et al. A diagnostic strategy to distinguish autoimmune pancreatitis from pancreatic cancer. Clinical Gastroenterology and Hepatology. 2009;7:1097.
- Sah RP, et al. Autoimmune pancreatitis: An update on classification, diagnosis, natural history and management. Current Gastroenterology Reports. 2012;14:95.
- Greenberger NJ. Autoimmune pancreatitis. http://www.uptodate.com/home. Accessed Oct. 22, 2014.
- O'Reilly DA, et al. Review of the diagnosis, classification and management of autoimmune pancreatitis. World Journal of Gastrointestinal Pathophysiology. 2014;5:71.
- Pancreatic cancer symptoms. National Cancer Institute. http://www.cancer.gov/cancertopics/wyntk/pancreas/page5. Accessed Oct. 23, 2014.
- What are pancreatic enzymes? Pancreatic Cancer Action Network. http://www.pancan.org/section-facing-pancreatic-cancer/learn-about-pan-cancer/diet-and-nutrition/pancreatic-enzymes/. Accessed Oct. 27, 2014.
- Golden AK. Decision Support System. Mayo Clinic, Rochester, Minn. Sept. 26, 2014.
- Kamisawa T, et al. Recent advances in autoimmune pancreatitis: Type 1 and type 2. Gut. 2013;62:1373.
- Hart PA, et al. Treatment of relapsing autoimmune pancreatitis with immunomodulators and rituximab: The Mayo Clinic experience. Gut. 2013;62:1607.
- Shimosegawa T, et al. International consensus diagnostic criteria for autoimmune pancreatitis: Guidelines of the International Association of Pancreatology. Pancreas. 2011;40:352.
- Khandelwal A, et al. Recent advances in the diagnosis and management of autoimmune pancreatitis. American Journal of Roentgenology. 2014;202:1007.
- Chari ST (expert opinion). Mayo Clinic, Rochester, Minn. Nov. 18, 2014.