This protocol covers first-line management of confirmed Type 1 AIP — lymphoplasmacytic sclerosing pancreatitis — as defined by international consensus diagnostic criteria. Accurate subtype classification is essential before initiating therapy, as Type 1 AIP and Type 2 AIP (IDCP) have distinct natural histories and management pathways.

Clinical Scenario

Per the ICDC, AIP is classified as Type 1 (lymphoplasmacytic sclerosing pancreatitis) or Type 2 (idiopathic duct centric pancreatitis, IDCP). This protocol applies exclusively to confirmed Type 1 AIP. The two subtypes differ significantly in natural history, diagnostic criteria, and management — this distinction must be established before treatment is selected.

First-Line Treatment Approach

Steroid therapy is the recommended first-line treatment for confirmed Type 1 AIP. The full regimen — including the specific agent, dosing schedule, and taper strategy — is defined in the complete 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.
ICDC recommend treating mild jaundice with no signs of infection with steroid therapy alone.

DOI: 10.1016/j.cgh.2019.04.052

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Treatment of Type 1 Autoimmune Pancreatitis (Lymphoplasmacytic Sclerosing Pancreatitis)

Clinical Scenario

First-Line Treatment Approach

Treatment Goal

References