Purpose of review: To summarize the existing knowledge of autoimmune pancreatitis (AIP) and to review the progress made in the diagnosis and treatment of AIP in the past year.
Recent findings: The term 'AIP' appears to encompass at least two distinct subtypes, type 1 and type 2. Type 1 AIP is the pancreatic manifestation of a systemic fibroinflammatory disease called immunoglobulin G4-associated systemic disease. Type 2 AIP affects younger patients, does not have a gender predilection and is associated with normal serum immunoglobulin G4 levels. Existing criteria are geared toward diagnosis of type 1; type 2 AIP can be definitively diagnosed only on pancreatic histology. Both subtypes respond to corticosteroid therapy. However, there are no standardized protocols for initial treatment or management and prevention of relapses in AIP. A novel antibody for AIP has recently been identified and its performance needs validation from other centers. Newly published strategies for differentiating AIP from pancreatic cancer are available.
Summary: AIP is a rare disease whose recognition and understanding are evolving. Much needs to be elucidated with regard to its cause, pathogenesis, treatment of relapse and long-term outcomes. A multidisciplinary team, familiar with the disease, is critical in making the correct diagnosis.