Autoimmune Pancreatitis

Overview

What is the pancreas?

The pancreas is a large gland in the abdomen located behind the stomach and next to the upper part of the small intestine (the duodenum).

Liver, Gallbladder, Esophagus, Stomach, Pancreas, Duodenum

The pancreas has two main jobs:

  1. It sends powerful digestive enzymes into the small intestine to help with the digestion of carbohydrates, proteins and fat.
  2. It releases the hormones insulin and glucagon into the bloodstream. These hormones are involved in the metabolism of blood glucose (sugar), which regulates how the body stores and uses food for energy.

What is pancreatitis?

Pancreatitis is a rare disease in which the pancreas becomes inflamed (swollen). Damage to the pancreas occurs when the digestive enzymes begin attacking the pancreas.

In very severe cases, pancreatitis can cause bleeding into the pancreas, serious tissue damage, infection, and cyst formation. Severe pancreatitis can also cause damage if enzymes and toxins are released into the bloodstream, which can harm other vital organs such as the heart, lungs and kidneys.

What is autoimmune pancreatitis?

Autoimmune pancreatitis (AIP) is an autoimmune disorder, in which the body’s immune system attacks its own healthy cells. There are two types of AIP:

  • Type 1, which is more common, affects the pancreas and other organs in the body, including the liver and gallbladder.
  • Type 2 associated with inflammatory bowel disease.

How common is autoimmune pancreatitis?

AIP occurs in fewer than one in 100,000 people. It usually develops in people who are between the ages of 50 and 60, but can occur sooner. AIP affects men twice as often as women. The causes of AIP are not known.

Symptoms and Causes

What are the signs and symptoms of autoimmune pancreatitis?

The signs and symptoms of AIP are similar to pancreatic cancer, but AIP is not cancer.

AIP does not always cause symptoms. When it does, the main symptom is jaundice (yellowing of the skin and whites of the eyes). Other signs and symptoms can resemble symptoms of pancreatic cancer.

Symptoms of AIP include:

  • Mass or growth in the pancreas
  • Stomach pain
  • Weight loss
  • Back pain
  • Fatigue (extreme tiredness)

Diagnosis and Tests

How is autoimmune pancreatitis diagnosed?

It is important to diagnose AIP correctly to rule out cancer and receive the right treatment. A doctor diagnoses AIP with several tests:

  • Blood tests can detect abnormal levels of a substance called serum IgG4, which can be a sign of AIP.
  • Imaging tests include:
    • Ultrasound and computed axial tomography scan (CAT scan), which can produce images of the pancreas so that abnormalities may be seen;
    • Esophagogastroduodenoscopy with ultrasound (images of the pancreas are obtained from inside the stomach and intestine using sound waves).
  • In some cases, doctors use a biopsy to rule out pancreatic cancer. In a biopsy, the doctor uses a small needle to remove a small amount of tissue from the pancreas for examination under a microscope.

To help make a definite diagnosis, your doctor may give you a trial dose of corticosteroids (medications that reduce inflammation). If the levels of serum IgG4 return to normal, it usually confirms the condition as AIP.

Management and Treatment

How is autoimmune pancreatitis managed or treated?

AIP is treated with corticosteroids to reduce inflammation. Treatment usually begins with a high dose of steroids, and then the dose is tapered down over time.

AIP doesn't usually require any other treatment. In some people, the disorder goes away without treatment. It is not uncommon for Type 1 AIP to relapse (return) when corticosteroid therapy stops. In that case, your doctor may recommend either another round of corticosteroid therapy or alternative steroid sparring strategies.

What complications are associated with autoimmune pancreatitis?

AIP sometimes occurs along with other autoimmune conditions such as rheumatoid arthritis and inflammatory bowel disease.

Prevention

Can autoimmune pancreatitis be prevented?

Because the causes of AIP are not known, it cannot be prevented.

Outlook / Prognosis

What is the prognosis (outlook) for people who have autoimmune pancreatitis?

AIP does not affect life expectancy. Some people need to continue steroid therapy for several years or for the rest of their lives to keep symptoms from returning. With successful management, people with the disorder can live active, healthy lives.

Living With

When should I call the doctor about symptoms of autoimmune pancreatitis?

Contact your doctor if you have jaundice or the other signs and symptoms of AIP.

What questions should I ask my doctor about autoimmune pancreatitis?

If you have AIP, you may want to ask your doctor:

  • How will you confirm it is not cancer?
  • How serious is AIP?
  • What risks or side effects are associated with steroid treatment?
  • How will AIP affect my lifestyle?

When can I go back to my regular activities after I have been treated for autoimmune pancreatitis?

Steroid therapy for AIP usually relieves symptoms within a few weeks. Most people can go back to their usual activities once their symptoms are gone.

Last reviewed by a Cleveland Clinic medical professional on 08/04/2019.

References

  • National Institute of Diabetes and Digestive and Kidney Diseases. Pancreatitis. (https://www.niddk.nih.gov/health-information/digestive-diseases/pancreatitis/all-content) Accessed 8/5/2019.
  • American Pancreatic Association. Prognosis and Long Term Outcomes of Autoimmune Pancreatitis. (https://www.pancreapedia.org/reviews/prognosis-and-long-term-outcomes-of-autoimmune-pancreatitis) Accessed 8/5/2019.
  • Hart PA, Yoh Z, and Suresh TC. Recent Advances in Autoimmune Pancreatitis.. Gastroenterology 2015;149:39–51.
  • Kim E, Voaklander R, Kasmin FE, et al. Autoimmune Pancreatitis: A Multiorgan Disease Presenting a Conundrum for Clinicians in the West. _Gastroenterology & Hepatology. _September 2015, Volume 11, Issue 9.
  • U.S. National Library of Medicine/Medline Plus. Pancreatitis. (https://medlineplus.gov/pancreatitis.html) Accessed 8/5/2019.
  • American Gastroenterological Association. Treatment of Autoimmune Pancreatitis: When to offer Immunomodulators or Rituximab. (https://www.gastro.org/news/treatment-of-autoimmune-pancreatitis-when-to-offer-immunomodulators-or-rituximab) Accessed 8/5/2019.

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