Autoimmune Pancreatitis: An Update

Authors and Disclosures

Aravind Sugumar,  Suresh Chari,
Mayo Clinic College of Medicine, Division of Gastroenterology and Hepatology, 200 First St SW, Rochester, MN, USA

Finanacial & competing interests disclosure
The authors have no relevant affiliations or financial involvements with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

No writing assistance was utilized in the production of this manuscript.

Key Issues

• Autoimmune pancreatitis (AIP) is a rare but distinct subtype of chronic pancreatitis.
  
  

• AIP is best described as the pancreatic manifestation of a systemic fibroinflammatory conditions. The systemic disease of which AIP is a part has been called IgG4-related systemic diseases.
  
  

• AIP mimics pancreatic cancer and it is critically important to distinguish between the two diseases.
  
  

• AIP is predominantly seen in males over the age of 50 years.
  
  

• Obstructive jaundice is the most common presenting symptom.
  
  

• Diagnosing AIP can be a challenge.
  
  

• Multiple diagnostic criteria have been proposed. For example, the Japanese Pancreas Society (2002 and 2006) and Histology, Imaging, Serology, Other Organ Involvement and Response to therapy (HISORt).
  
  

• There is a classic presentation, which includes obstructive jaundice, sausage-shaped pancreatic enlargement with a low-density rim, serum IgG4 elevation and dramatic response to steroids, but AIP can present in more subtle ways without classical symptoms.
  
  

• There is often a dramatic response to steroids.
  
  

• Relapses are quiet common and may need a prolonged course of steroids.
  
  

• As the collective experience with this condition increases, better diagnostic and therapeutic protocols will emerge.