Overview of the management of patients with autoimmune pancreatitis

AIP: autoimmune pancreatitis; CT: computed tomography; MRI: magnetic resonance imaging; IgG4-RD: immunoglobulin G4-related disease; IgG4-SC: IgG4-sclerosing cholangitis.
* Patients are advised to report new AIP-related symptoms and require periodic clinical evaluation, laboratory testing, and abdominal imaging.
¶ Refer to UpToDate content on autoimmune pancreatitis for additional details.
Δ Other steroid-sparing agents such as thiopurines are poorly effective as single agents for induction of remission.
◊ The lack of response to a glucocorticoid trial warrants referral for surgical exploration for pancreatic cancer.
§ Options include low-dose prednisone, thiopurines (azathioprine, 6-mercaptopurine), or rituximab. The choice of agent depends upon individual patient factors (presence of IgG4 RD) and cost/insurance considerations, and availability. We use azathioprine as a first line of therapy for maintenance treatment of AIP and use rituximab in patients with IgG4-RD or resistance to or side effects from treatments including glucocorticoids and immunomodulators.