Autoimmune pancreatitis
ICD-10 K86.1 · ICD-11 DC33

Treatment of Autoimmune Pancreatitis Presenting as Idiopathic Duct-Centric Pancreatitis (Type 2 AIP / IDCP)

Not all autoimmune pancreatitis is the same. When a patient is diagnosed with idiopathic duct-centric pancreatitis — the entity classified as type 2 AIP — the clinical picture, natural history, and management differ meaningfully from type 1 disease. The protocol below addresses this specific subtype.

Clinical scenario

The international consensus diagnostic criteria (ICDC) distinguish type 1 AIP (lymphoplasmacytic sclerosing pancreatitis) from type 2 AIP (idiopathic duct-centric pancreatitis, IDCP). Because of significant differences in natural history, diagnostic criteria, and management between the two subtypes, type 2 AIP is designated specifically as IDCP. This protocol applies to confirmed or suspected IDCP — not type 1 autoimmune pancreatitis.

Treatment approach (partial overview)

Initial management of IDCP involves a course of oral corticosteroid therapy, followed by a structured taper.

The complete regimen, taper schedule, and monitoring parameters are in the full protocol.

References

  • The international consensus diagnostic criteria (ICDC) classify AIP as type 1 AIP or lymphoplasmacytic sclerosing pancreatitis, type 2 AIP or idiopathic duct centric pancreatitis (IDCP), or AIP not otherwise specified.
  • Because of the significant differences among type 1 and type 2 AIP with regard to natural history, diagnostic criteria, and management, the term AIP is restricted to lymphoplasmacytic sclerosing pancreatitis, whereas type 2 AIP is termed IDCP.
  • Prednisone (40 mg/day x 4 weeks) and taper by 5 mg per week.

DOI: 10.1016/j.cgh.2019.04.052

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