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Autoimmune Pancreatitis

Last updated May 3, 2018

Approved by: Maulik P. Purohit MD MPH

Autoimmune Pancreatitis affects the pancreas, and may also affect the bile ducts, salivary glands, kidneys, and lymph nodes. The condition is thought to occur when the immune system mistakenly begins to attack these healthy body tissues, glands, and organs.

What are the other Names for this Condition? (Also known as/Synonyms)

  • Idiopathic Duct-Centric Pancreatitis
  • IgG4-Related Pancreatitis
  • Lymphoplasmocytic Sclerosing Pancreatitis

What is Autoimmune Pancreatitis? (Definition/Background Information)

  • Autoimmune Pancreatitis affects the pancreas, a gland behind the stomach and in front of the spine, and can also affect the bile ducts, salivary glands, kidneys, and lymph nodes
  • It is thought to occur when the immune system mistakenly begins to attack these healthy body tissues, glands, and organs
  • Common signs and symptoms include painless jaundice, weight loss, and non cancerous masses in the pancreas and other organs
  • Treatment often involves corticosteroids. The condition may recur following treatment, and require additional therapy

(Source: Autoimmune Pancreatitis; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

Autoimmune Pancreatitis can be classified as types 1 and 2:

  • Type 1, or IgG4-Related Pancreatitis, is known to affect many different organs and systems within the body, which may include the salivary glands, hepatic bile ducts, kidneys and lymph nodes, in addition to the pancreas
  • Type 2, or Idiopathic Duct-Centric Pancreatitis, specifically affects the pancreas. However, it has been reported that about 30% of individuals affected by this type of Autoimmune Pancreatitis also may have inflammatory bowel disease (IBD)

Who gets Autoimmune Pancreatitis? (Age and Sex Distribution)

  • Autoimmune Pancreatitis is a rare disorder that affects older individuals
  • Both males and females may be affected
  • Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Autoimmune Pancreatitis? (Predisposing Factors)

The risk factors for Autoimmune Pancreatitis are different for the two types and are as follow:

  • Autoimmune Pancreatitis Type 1:
    • Male gender
    • Age over 60 years
  • Autoimmune Pancreatitis Type 2:
    • Inflammatory bowel disease
    • Age over 40 years

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Autoimmune Pancreatitis? (Etiology)

  • Autoimmune Pancreatitis is caused when an individual’s immune system mistaken;y attacks his/her own body tissues, glands, and organs
  • The exact reason for the autoimmune response in Autoimmune Pancreatitis is not known

What are the Signs and Symptoms of Autoimmune Pancreatitis?

Autoimmune Pancreatitis may be asymptomatic. If symptoms do occur, they may differ in type and severity among affected individuals. The following are some known signs and symptoms of the condition:

  • Loss of appetite
  • Nausea and vomiting
  • Unintended weight loss
  • Pain in upper abdomen; pain in the middle of back
  • Weakness, lethargy
  • Jaundice
  • Dark urine
  • Stools that are pale; stools that float and are difficult to flush

How is Autoimmune Pancreatitis Diagnosed?

Autoimmune Pancreatitis is diagnosed on the basis of the following information:

  • Complete physical examination
  • Thorough medical history evaluation
  • Assessment of signs and symptoms
  • Laboratory tests
  • Imaging studies
  • Biopsy studies, if necessary
  • Differential diagnosis, to confirm Autoimmune Pancreatitis and rule-out pancreatic cancer; the signs and symptoms of Autoimmune Pancreatitis often mimic those of pancreatic cancer

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Autoimmune Pancreatitis?

The complications of Autoimmune Pancreatitis may include:

  • Malfunction of pancreas, leading to low production of enzymes and hormones. This may lead to the following conditions:
  • Diabetes
  • Metabolic bone disease
  • Deficiencies in vitamins and minerals
  • Pancreatic stones
  • Obstructive jaundice

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Autoimmune Pancreatitis Treated?

The treatment for Autoimmune Pancreatitis may include the following:

  • Administration of steroids
  • Immunotherapy
  • Immunosuppressants such as rituximab
  • Biliary stenting, to drain the bile ducts and relieve obstructive jaundice
  • Insulin treatment for diabetes, if it caused by insulin insufficiency
  • Mineral and vitamin supplementations, to address deficiencies

How can Autoimmune Pancreatitis be Prevented?

The exact cause of Autoimmune Pancreatitis is not known, and hence, no guidelines or methods are available for its prevention.

  • Individuals with inflammatory bowel disease or other autoimmune disorders should seek prompt medical treatment to help detect Autoimmune Pancreatitis early and avoid complications
  • Active research is currently being performed to explore the possibilities for treatment and prevention of disorders such as Autoimmune Pancreatitis
  • Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Autoimmune Pancreatitis? (Outcomes/Resolutions)

  • The prognosis of Autoimmune Pancreatitis is dependent upon the severity of the signs and symptoms and associated complications, if any
  • Individuals with mild conditions have better prognosis than those with severe symptoms and complications
  • Typically, the prognosis may be assessed on a case-by-case basis

Additional and Relevant Useful Information for Autoimmune Pancreatitis:

The following DoveMed website link is a useful resource for additional information:


What are some Useful Resources for Additional Information?

References and Information Sources used for the Article:

Helpful Peer-Reviewed Medical Articles:

Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: May 3, 2018
Last updated: May 3, 2018