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.

Autoimmune pancreatitis (AIP) is difficult to diagnose. Often, it doesn't cause any symptoms. When it does, its symptoms and signs are very similar to those of pancreatic cancer.

Pancreatic cancer symptoms can include:

  • Dark urine
  • Pale stools or stools that float in the toilet
  • Yellow skin and eyes (jaundice)
  • Pain in your upper abdomen or middle part of your back
  • Nausea and vomiting
  • Weakness or extreme tiredness
  • Loss of appetite or feelings of fullness
  • Weight loss for no known reason

The most common sign of autoimmune pancreatitis, present in about 80 percent of people, is painless jaundice, caused by blocked bile ducts. AIP can also cause weight loss. Many people with autoimmune pancreatitis have masses in the pancreas and other organs, which can be misdiagnosed as cancer.

The two types of AIP occur with different frequency in different parts of the world. In the United States, about 80 percent of people with autoimmune pancreatitis have type 1.

People with type 1 autoimmune pancreatitis often:

  • Are over age 60
  • Are male
  • Have multiple organ involvement including the bile ducts (IgG4-related sclerosing cholangitis), the salivary glands (IgG-related sialadenitis), or the lungs (IgG4-related pulmonary disease)
  • Have retroperitoneal fibrosis (RPF), which is the formation of extra fibrous tissue in the area behind the stomach and intestine
  • Have lymph node enlargement (lymphadenopathy), low thyroid (hypothyroidism) or kidney disease

People with type 2 autoimmune pancreatitis:

  • Are often over age 40 (one or two decades younger than those with type 1)
  • Are as likely to be female as male
  • Have a 30 percent chance of having inflammatory bowel disease, such as ulcerative colitis

When to see a doctor

Autoimmune pancreatitis often doesn't cause any symptoms. See your doctor, however, if you experience unexplained weight loss, abdominal pain, jaundice, or other signs and symptoms that bother you.

Doctors don't know what causes autoimmune pancreatitis, but as in other autoimmune diseases, the body's immune system attacks healthy body tissue.

Autoimmune pancreatitis can cause a variety of complications.

  • Pancreatic insufficiency. AIP may affect the ability of your pancreas to make enough enzymes. Signs and symptoms may include diarrhea, weight loss, metabolic bone disease, and vitamin or mineral deficiency.
  • Diabetes. Because the pancreas is the organ that produces insulin, damage to it may cause diabetes, and you may need treatment with oral medication or insulin.
  • Pancreatic calcifications or stones.

Treatments for autoimmune pancreatitis, such as long-term steroid use, also can cause complications. However, even with these complications, people who are treated for autoimmune pancreatitis have a normal life expectancy.

There is no established association between AIP and pancreatic cancer.

Start by seeing your primary care doctor if you have signs or symptoms that worry you. If your doctor suspects you may have autoimmune pancreatitis, you may be referred to a gastroenterologist.

Because appointments can be brief and because there's often a lot of ground to cover, it's a good idea to be well-prepared. Here's some information to help you get ready and to help you know what to expect from your doctor.

What you can do

  • Be aware of any pre-appointment restrictions. When you make your appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
  • Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
  • Write down key personal information, including any major stresses or recent life changes.
  • Make a list of all medications and any vitamins or supplements that you're taking.
  • Consider taking along a family member or friend. Sometimes it can be difficult to remember all the information provided during an appointment. Someone who accompanies you may remember something that you missed or forgot.
  • Write down questions to ask your doctor.

Your time with the doctor is limited. You may feel less rushed if you make a list of questions you want to ask at your appointment. Here are some possible questions about autoimmune pancreatitis:

  • Can you explain my test results to me?
  • Will I need more tests?
  • How far has my disease progressed?
  • Will it come back after initial treatment?
  • Do I need lifelong treatment to prevent relapse?
  • How should I be followed for relapse?
  • How severe is the damage to my pancreas?
  • What treatments can relieve my signs and symptoms?
  • What are the potential side effects of each treatment?
  • What signs and symptoms signal that my condition is worsening and I need to make another appointment?
  • What signs and symptoms of complications should I watch for?
  • I have other health conditions. How can I best manage them together?
  • Are there any restrictions that I need to follow?
  • Are there any brochures or other printed material that I can take with me? What websites do you recommend?

In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask questions that come up during your appointment.

What to expect from your doctor

Your doctor is likely to ask you a number of questions. Being ready to answer them may allow more time later to cover points you want to address. Your doctor may ask:

  • When did you begin experiencing symptoms?
  • Do you have abdominal pain, dark urine, pale stools, itching or weight loss?
  • Have your symptoms been continuous or occasional?
  • How severe are your symptoms?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?
  • Have you had these symptoms before?
  • Have you ever been diagnosed with pancreatitis?
  • Did you start any new medications before your symptoms began?

Autoimmune pancreatitis is difficult to diagnose, because its signs and symptoms are very similar to those of pancreatic cancer. However, an accurate diagnosis is extremely important. Undiagnosed cancer may result in delaying or not receiving necessary surgery. On the other hand, having unneeded surgery that is invasive and risky also should be avoided.

There are two types of autoimmune pancreatitis. Doctors will use a number of tests to determine if you have AIP and if so, which type you have.

People with AIP type 1 have:

  • One or more masses in the pancreas.
  • A high level of (elevated) serum IgG4 in the pancreas.
  • In some cases, evidence of the disease in other organs, called other organ involvement (OOI), such as masses or elevated serum IgG4.
  • A rapid response to treatment with steroids.
  • A likelihood of relapse if treatment is discontinued.

In contrast, people with AIP type 2 have:

  • No evidence of the disease other than in the pancreas.
  • No serum IgG4 elevation.
  • No OOI.
  • GELs. Granulocyte epithelial lesions (GELS) are a type of white blood cell (neutrophils) in the ducts of the pancreas, often causing destruction of the ducts.
  • Little likelihood of relapse after treatment is discontinued.

Because people with AIP type 2 don't have elevated IgG4 or other organ involvement, a definitive diagnosis requires a biopsy (pancreatic histology).

Tests

No single test or characteristic feature identifies autoimmune pancreatitis. Until recently, different diagnostic approaches around the world made the diagnosis more difficult. Researchers established the International Consensus Diagnostic Criteria (ICDC) in 2011:

  • Microscopic analysis of cells and tissues (histology)
  • Imaging, such as CT, MRI or ERCP
  • Serology (serum IgG4 levels)
  • Other organ involvement
  • Optionally, response to steroid therapy — usually a two-week steroid trial

Specific tests may include:

  • Imaging tests. Tests of your pancreas and other organs may include CT, MRI, endoscopic ultrasound (EUS) and endoscopic retrograde cholangiopancreatography (ERCP).
  • Blood tests. You will be tested for elevated levels of an immunoglobulin called IgG4, produced by your immune system. People with type 1 AIP, but not with type 2 AIP, will have highly elevated blood levels of IgG4.

    However a positive test doesn't necessarily mean that you have the disease. A small number of people who don't have autoimmune pancreatitis, including some with pancreatic cancer, also have high blood levels of IgG4.

  • Endoscopic core biopsy. In this test, pathologists analyze a sample of pancreatic tissue in the laboratory. AIP has a distinctive appearance that is easily recognized under a microscope. Doctors insert a small tube (endoscope) through the mouth into the stomach and, guided by ultrasound, remove some tissue from the pancreas using a special needle.

    The challenge is obtaining a sample of tissue large enough to analyze, rather than just a few cells. This procedure is not widely available.

  • Steroid trial. Because autoimmune pancreatitis is the only pancreatic disorder known to respond to steroids, doctors sometimes use a trial course of this drug to confirm a diagnosis.
  • Biliary stenting. Before starting medication, sometimes doctors will insert a tube to drain the biliary ducts (biliary stenting) in people with symptoms of obstructive jaundice. Often, though, jaundice improves with steroid treatment alone. Sometimes drainage is recommended if the diagnosis is uncertain.
  • Steroids. Symptoms of autoimmune pancreatitis often improve after a short course of prednisolone (Prelone). Many people respond quickly, even dramatically. Sometimes people get better without any treatment.
  • Immunosuppressants and immunomodulators. About 30 to 50 percent of the time, AIP returns, requiring additional treatment, sometimes long term. To help reduce the serious side effects associated with extended steroid use, doctors often add drugs that suppress or modify the immune system, sometimes called steroid-sparing medications, to the treatment. You may be able to stop taking steroids altogether.

    Immunosuppresants and immunomodulators include mycophenolate (CellCept), mercaptopurine (Purinethol, Purixan), azathioprine (Imuran, Azasan) and rituximab (Rituxan). In general, they have been used in small sample groups and long-term benefits are still being studied.

  • Treatment for pancreatic insufficiency. If you have insufficient pancreatic enzymes, you may need supplementary enzymes. If you need supplements, your doctor will prescribe pancreatic enzymes (Creon, Viokace, Pancreaze). The over-the-counter versions of these drugs are not regulated by the Food and Drug Administration.
  • Treatment for diabetes. If you have diabetes, you will need appropriate treatment.
  • Monitoring of other organ involvement (OOI). Type 1 AIP often is associated with other organ involvement, including enlarged lymph nodes and salivary glands, scarring of the bile ducts, liver inflammation, and kidney disease. Although these signs may lessen or disappear completely with steroid therapy, your doctor will continue to monitor you.
Dec. 20, 2014