Disease: Autoimmune pancreatitis

Appointments & care

At Mayo Clinic, we take the time to listen, to find answers and to provide you the best care.

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.

Source: http://www.mayoclinic.com

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.

Source: http://www.mayoclinic.com

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

Source: http://www.mayoclinic.com

Appointments & care

At Mayo Clinic, we take the time to listen, to find answers and to provide you the best care.

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.

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.

Source: http://www.mayoclinic.com

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.

Source: http://www.mayoclinic.com

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