Autoimmune pancreatitis is a chronic inflammation thought to be caused by the body's immune system attacking the pancreas. The disease affects not only the pancreas, but also the bile ducts in the liver, as well as the salivary glands, kidneys and lymph nodes. One type of the disease mainly affects men in their 50s and 60s; a second type can occur in children as young as 12.

Autoimmune pancreatitis is a rare disease and can be mistakenly diagnosed as pancreatic cancer. Both conditions have similar signs and symptoms, but very different treatments, so it is crucial to distinguish one from another. Autoimmune pancreatitis is treated with steroids, which improve the condition in many people, often dramatically.

  • Experience. Mayo Clinic treats close to 2,000 people each year for pancreatitis, including autoimmune pancreatitis.
  • Diagnosis leader. Mayo Clinic doctors have led efforts to establish diagnostic guidelines for autoimmune pancreatitis, to help distinguish the disease from pancreatic cancer and prevent people from undergoing unnecessary surgery. Mayo specialists are experts at endoscopic core needle biopsy, a procedure that allows them to obtain samples of pancreatic tissue for analysis without surgery.
  • Teamwork. An experienced team of specialists in pancreatic disease, including a gastroenterologist, endoscopist, pathologist and radiologist, works together with other specialists to provide accurate diagnosis and comprehensive treatment.
  • Active research. Mayo Clinic has played a major role in identifying and describing this newly recognized disorder and in evaluating treatments. Mayo researchers also identified a type of autoimmune pancreatitis that can develop in children as young as 12 years of age. You can benefit from new advances that Mayo is helping to discover.

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Specialists in digestive diseases (gastroenterology) treat adults who have autoimmune pancreatitis.

For appointments or more information, call the Central Appointment Office at 800-446-2279 (toll-free) 8 a.m. to 5 p.m. Mountain Standard Time, Monday through Friday or complete an online appointment request form.

Specialists in digestive diseases (gastroenterology) treat adults who have autoimmune pancreatitis.

For appointments or more information, call the Central Appointment Office at 904-953-0853 8 a.m. to 5 p.m. Eastern time, Monday through Friday or complete an online appointment request form.

Specialists in the Pancreas Clinic treat adults who have autoimmune pancreatitis.

For appointments or more information, call the Central Appointment Office at 507-538-3270 7 a.m. to 6 p.m. Central time, Monday through Friday or complete an online appointment request form.

See information on patient services at the three Mayo Clinic locations, including transportation options and lodging.

Autoimmune pancreatitis (AIP) can be particularly challenging to diagnose because the way it presents itself closely resembles pancreatic cancer. The most common sign, present in about 80 percent of people, is painless jaundice, caused by blocked bile ducts. AIP can also cause weight loss. Masses in the pancreas and other organs of many people with autoimmune pancreatitis can be misdiagnosed as cancer.

Pancreatic specialists at Mayo Clinic are leading efforts to standardize diagnostic guidelines so that the disorder can be consistently and correctly identified and unnecessary surgery avoided.

No single test or characteristic feature identifies autoimmune pancreatitis. Mayo's approach to diagnosis comprises the following:

  • Imaging tests. Imaging of the pancreas and other organs is usually the first step in diagnosis. Mayo Clinic radiologists have extensive experience evaluating autoimmune pancreatitis using advanced technologies, such as dual-phase helical CT scans and MRI, which produce superior images of the pancreas, bile ducts and liver. It is essential to have images read by radiologists with expertise in pancreatic disorders.
  • Blood tests. Elevated levels of an antibody called IgG4 are a common feature of autoimmune pancreatitis. These antibodies are produced by your immune system. But a positive test doesn't mean that you have the disease. Studies at Mayo Clinic have shown that a small number of people who don't have autoimmune pancreatitis, including some with pancreatic cancer, also have high blood levels of IgG4 antibodies.
  • Endoscopic core biopsy. Pathologists analyze a sample of pancreatic tissue in the laboratory to help confirm the diagnosis of autoimmune pancreatitis. The disease has a distinctive appearance that is easily recognized under a microscope. The challenge is obtaining a sample of whole tissue large enough to analyze. Mayo Clinic specialists are often able to get a suitable sample by inserting a small tube (endoscope) through the mouth into the stomach and, guided by ultrasound, removing some tissue from the pancreas (biopsy) using a special needle. Mayo Clinic is one of the few medical centers in the world performing core biopsies of the pancreas.
  • Steroid trial. Because autoimmune pancreatitis is the only pancreatic disorder known to respond to corticosteroids, Mayo doctors sometimes use a trial course of this drug to confirm a diagnosis. If your signs and symptoms improve — for instance, your IgG4 level drops or a mass in your pancreas shrinks — you are considered to have autoimmune pancreatitis. However, this approach should be used only by doctors thoroughly familiar with the disease, as steroids give a general feeling of well-being even in people with cancer. Also, elevation of IgG4 not associated with AIP may also decrease with steroid use, giving a false sense of reassurance.

Symptoms of autoimmune pancreatitis often improve after a short course of corticosteroids. Many people respond quickly, even dramatically. Some people get better without any treatment. However, about 30 to 40 percent of the time, the disease returns (relapses), requiring additional treatment, sometimes long term. To help reduce the serious side effects associated with extended corticosteroid use, Mayo Clinic doctors add steroid-sparing medications to the treatment regimen. These drugs allow you to stop steroids altogether.

Autoimmune pancreatitis can be associated with other organ involvement, including enlarged lymph nodes and salivary glands, scarring of the bile ducts, liver inflammation, and kidney disease. Although these manifestations may diminish or disappear completely with steroid therapy, your treatment team will continue to monitor you for recurring problems. Other Mayo specialists are available to help manage these problems.

Mayo Clinic doctors have been leaders in recognizing and classifying autoimmune pancreatitis and continue to investigate better methods to diagnose and treat the disorder. Some current inquiries center on:

  • Value of IgG4 in diagnosis
  • Other organ involvement
  • Ideal length of steroid treatment

Mayo Publications

See a list of publications by Mayo Clinic doctors on autoimmune pancreatitis on PubMed, a service of the National Library of Medicine.

Jan. 12, 2012