Autoimmune Pancreatitis

Autoimmune pancreatitis (AIP) is a rare and chronic inflammatory disease of the pancreas that is characterized by an autoimmune response, where the body's immune system mistakenly attacks the pancreas.

Types of Autoimmune Pancreatitis

There are two main types of AIP:

  • Type 1 AIP: This type is characterized by elevated levels of IgG4, a type of antibody, and is often associated with other autoimmune disorders such as Sjögren's syndrome and rheumatoid arthritis.
  • Type 2 AIP: This type is not associated with elevated IgG4 levels and is more commonly seen in patients with inflammatory bowel disease.

Causes and Risk Factors

The exact cause of AIP is not fully understood, but it is believed to involve a combination of genetic and environmental factors that trigger an autoimmune response. Risk factors for developing AIP include:

  • Family history of autoimmune disorders
  • Other autoimmune conditions such as Sjögren's syndrome or rheumatoid arthritis
  • Inflammatory bowel disease

Symptoms

The symptoms of AIP can vary, but common presentations include:

  • Abdominal pain
  • Nausea and vomiting
  • Weight loss
  • Fatigue
  • J jaundice (yellowing of the skin and eyes)

Diagnosis

Diagnosing AIP can be challenging, but a combination of the following tests and procedures may be used:

  • Imaging studies such as CT or MRI scans to visualize the pancreas
  • Blood tests to measure IgG4 levels and other markers of inflammation
  • Endoscopic ultrasound (EUS) to evaluate the pancreas and bile ducts
  • Biopsy of the pancreas to confirm the diagnosis

Treatment

Treatment for AIP typically involves:

  • Corticosteroids to reduce inflammation and suppress the immune system
  • Immunomodulatory medications such as azathioprine or mercaptopurine to modify the immune response
  • Pain management with analgesics or other medications
  • Nutritional support to manage malabsorption and weight loss

Prognosis

The prognosis for AIP is generally good, with most patients responding well to treatment. However, some patients may experience relapses or develop complications such as pancreatic insufficiency or bile duct strictures.

Article last updated on: 6th June 2025.
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