Description

Autoimmune pancreatitis is an inflammation of the pancreas. It can be caused by the immune system attacking the pancreas. Autoimmune pancreatitis is also called AIP. Two subtypes of AIP are now recognized, type 1 and type 2.

Type 1 AIP is called IgG4-related disease (IgG4-RD). This type 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 around a third of people with diabetes type 2 AIP have been associated with inflammatory bowel disease.

Type 1 AIP can be misdiagnosed as pancreatic cancer. The two conditions have overlapping symptoms, but very different treatments, so it is very important to distinguish one from the other.

Symptoms

Autoimmune pancreatitis, also called AIP, it is difficult to diagnose. Often, it does not cause any symptoms. The symptoms of type 1 AIP are similar to those of pancreatic cancer.

Pancreatic cancer symptoms can include:

  • Dark-colored urine.
  • Pale stools or stools that float in the toilet.
  • Yellow discoloration of the skin and eyes called jaundice.
  • Pain in the 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 type 1 AIP is painless jaundice. Approximately 80% of people with diabetes type 1 AIP has painless jaundice. This is caused by the obstruction of the bile ducts. People with diabetes type 2 AIP can have repeated episodes of acute pancreatitis. Pain in the upper abdomen, a common symptom of cancer of the pancreas, is often absent in autoimmune pancreatitis.

The differences between type 1 and type 2 AIP are:

  • In type 1 AIP , the disease can affect other organs in addition to the pancreas. Type 2 AIP only affects the pancreas. Type 2 disease is also associated with another autoimmune disease called inflammatory bowel disease.
  • Type 1 AIP mainly affects men in the sixth to seventh decade of life.
  • Type 2 AIP affects both men and women equally, and a younger age of onset compared with type 1 AIP .
  • Type 1 AIP is more likely to relapse after treatment is discontinued.

When to see a doctor

Autoimmune pancreatitis often causes no symptoms. Consult a health care professional, however, if you have unexplained weight loss, abdominal pain, jaundice or other symptoms that worry you.

Causes

Experts do not know what causes autoimmune pancreatitis, but it is believed that it is caused by the body's immune system attacks healthy body tissue. This is known as an autoimmune disease.

Risk factors

The two types of AIP occur with different frequency in different parts of the world. In the united States, approximately 80% of people with autoimmune pancreatitis, also called AIP, type 1.

People with diabetes type 1 AIP often:

  • Are over 60 years of age.
  • They are men.

People with diabetes type 2 AIP :

  • They are often one or two decades younger than those with type 1 diabetes.
  • It is as likely to be female as male.
  • Are more likely to have inflammatory bowel disease such as ulcerative colitis.

Complications

Autoimmune pancreatitis can cause a variety of complications.

  • Exocrine insufficiency of the pancreas. AIP can affect the ability of the pancreas to produce enough enzymes. Symptoms may include diarrhea, weight loss, metabolic bone disease, and deficiency of vitamins or minerals.
  • Diabetes. Because the pancreas is the organ that produces insulin, the damage that can cause diabetes. You may need treatment with oral medications or insulin.
  • The pancreas and the bile duct narrowing, called stenosis.
  • Pancreatic calcifications or stones.

Treatments for autoimmune pancreatitis, as the long-term use of steroids, can cause complications. However, even with these complications, people who receive treatment for autoimmune pancreatitis have a typical life expectancy.

There is no established association between AIP and pancreatic cancer.

Diagnosis

Autoimmune pancreatitis is difficult to diagnose because its symptoms are very similar to those of pancreatic cancer. However, an accurate diagnosis is extremely important. Undiagnosed cancer may result in the delay or not receive the necessary treatment.

People with AIP tend to have a general enlargement of the pancreas, but can also have a mass in the pancreas. To clarify the diagnosis and determine the type of AIP , blood, and imaging tests are necessary.

Tests

There is not a single test or feature identifies autoimmune pancreatitis. It is recommended guidelines for the diagnosis, the use of a combination of images, blood tests, and the results of the biopsy.

Specific tests may include:

  • Imaging tests. The evidence of his pancreas, and other organs may include CT , MRI , endoscopic ultrasound (EUS) and endoscopic retrograde cholangiopancreatography (ERCP).
  • Blood tests.You can have a test of the elevation of immunoglobulin levels, called IgG4. IgG4 is produced by the immune system. People with type 1AIPoften have high levels of IgG4 in your blood. People with type 2AIPusually not. However, a positive result does not necessarily mean you have the illness. A small number of people who do not have autoimmune pancreatitis, including some with cancer of the pancreas, also have high levels of IgG4 in your blood.
  • Endoscopic biopsy.In this test, a professional medical doctor called a pathologist examines a sample of pancreatic tissue in the laboratory.AIPhas a distinctive look that can be easily recognized under a microscope by an expert pathologist. A thin tube called an endoscope is inserted through the mouth into the stomach, guided by ultrasound. A sample of tissue is removed from the pancreas with a special needle. The challenge is to obtain a tissue sample is large enough to study, instead of only a few cells. This procedure is not widely available, and the results may not be conclusive.
  • Steroid trial. Autoimmune pancreatitis usually responds to steroids; the professionals of the health, the use of a course of testing this drug in order to confirm a diagnosis. However, this strategy should ideally happen under the guidance of experts. Should be used sparingly and should only be done when there is a strong evidence to support a diagnosis of autoimmune pancreatitis. Response to corticosteroids is measured by the TC, and the improvement in the serum levels of IgG4.

Blood tests. You can have a test of the elevation of immunoglobulin levels, called IgG4. IgG4 is produced by the immune system. People with diabetes type 1 AIP often have high levels of IgG4 in your blood. People with diabetes type 2 AIP is usually not.

However, a positive result does not necessarily mean you have the illness. A small number of people who do not have autoimmune pancreatitis, including some with cancer of the pancreas, also have high levels of IgG4 in your blood.

Endoscopic biopsy. In this test, a professional medical doctor called a pathologist examines a sample of pancreatic tissue in the laboratory. AIP has a distinctive look that can be easily recognized under a microscope by an expert pathologist. A thin tube called an endoscope is inserted through the mouth into the stomach, guided by ultrasound. A sample of tissue is removed from the pancreas with a special needle.

The challenge is to obtain a tissue sample is large enough to study, instead of only a few cells. This procedure is not widely available, and the results may not be conclusive.

Treatment

  • Biliary Stent. Before you start the medication, sometimes doctors or other health professionals a tube is inserted to drain the bile ducts. This is called a biliary stent and is performed in people with symptoms of obstructive jaundice. Often, however, the jaundice, improves with treatment with steroids alone. Sometimes, the drain is recommended if the diagnosis is not certain. The tissue samples and cells of the bile duct can be taken at the time of the placement of the stent.
  • Steroids. The symptoms of autoimmune pancreatitis often improve after a short course of prednisolone or prednisone. Many people respond quickly, even dramatically. Sometimes people recover without treatment.
  • Immunosuppressants and immunomodulators.The disease can relapse. For the type 1AIP, the disease relapses around 30% to 50% of the time. For the type 2AIP, is relapse less than 10% of the time. Cases of relapse and require additional treatment, sometimes long-term. To help reduce the serious side effects associated with the widespread use of steroids, drugs that suppress or modify the immune system can be added to the treatment. These are sometimes called steroid-sparing medications. You may be able to stop taking steroids completely depending on your response to the steroid-sparing of the medicine. Immunosuppressants and immunomodulators include mycophenolate (CellCept), mercaptopurine (Purinethol, Purixan), azathioprine (Imuran, Azasan) and rituximab (Rituxan). In general, have been used in small-groups of the sample and the long-term benefits are still being studied.
  • Follow-up of the involvement of other organs. Type 1 AIP is often associated with involvement of other organs, including the enlargement of the lymph nodes and salivary glands, fibrosis of the bile ducts, inflammation of the liver, and kidney disease. Although these symptoms may lessen or go away completely with steroid therapy, your healthcare team will continue to monitor you.

Immunosuppressants and immunomodulators. The disease can relapse. For the type 1 AIP , the disease relapses around 30% to 50% of the time. For the type 2 AIP , the relapse less than 10% of the time. Cases of relapse and require additional treatment, sometimes long-term.

To help reduce the serious side effects associated with the widespread use of steroids, drugs that suppress or modify the immune system can be added to the treatment. These are sometimes called steroid-sparing medications. You may be able to stop taking steroids completely depending on your response to the steroid-sparing of the medicine.

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

Preparing for your appointment

Start by seeing your health care team if you have symptoms that concern you. If your health care team suspects that you may have autoimmune pancreatitis, you may be referred to a health professional who specializes in digestive problems, called a gastroenterologist or a health professional who specializes in issues related to the pancreas, called the pancreatologist.

What you can do

  • Be aware of any pre-appointment restrictions. When you make your appointment, be sure to ask if there is anything that 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 the medications and any vitamins or supplements you are 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 questions to ask their doctor or other health care professional.

His time with the professional of the health is limited. To make the most of the time, bring a list of questions you have to the appointment. Here are some of the possible questions about the autoimmune pancreatitis:

  • Can you explain the results of my test for me?
  • How can you be sure that I don't have cancer of the pancreas?
  • More evidence is needed?
  • Up to what point my disease progressed?
  • Will be coming back after the initial treatment?
  • I need a life-long treatment to prevent relapse?
  • What should I do if it comes back?
  • How serious is the damage to my pancreas?
  • What treatments can relieve the symptoms?
  • What are the possible side effects of each treatment?
  • What are the symptoms of the signal that my condition is getting worse and that I need to make another appointment?
  • What are the symptoms of the complications should I be aware?
  • I have other health conditions. How can I best manage them together?
  • Are there any restrictions that I need to follow?
  • Are there brochures or other printed material that I can take with me? What sites do you recommend?

In addition to the questions that you've prepared to ask your health care professional, do not hesitate to ask questions during your appointment.

What to expect from your doctor

Your healthcare provider is likely to ask several questions. Be ready to answer them may allow more time later to cover the points you want to address. Your health care professional may ask:

  • When did you begin experiencing symptoms?
  • Do you have abdominal pain, dark urine, pale stools, itching, or weight loss?
  • The symptoms been continuous or occasional?
  • How severe are the 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 medicine before your symptoms began?
Symptoms and treatment of Autoimmune pancreatitis