Autoimmune pancreatitis Management Indications for treatment Symptomatic patients Q Is the patient symptomatic related to pancreatic involvement (e.g. obstructive jaundice, abdominal pain, back pain)? Q Is the patient symptomatic related to involvement of other organs (e.g. jaundice due to bile duct stricture)? Yes to any Treat patients with AIP who are symptomatic due to pancreatic involvement (e.g., obstructive jaundice, abdominal pain, back pain), or other organ involvement (e.g., jaundice due to bile duct stricture). B No to all Indications for treatment Asymptomatic patients Q Does the patient have a persistent pancreatic mass on imaging? Q Does the patient have IgG4-related sclerosing cholangitis and persistent liver test abnormalities? No to all As some patients with AIP (about 10-25%) improve spontaneously without intervention or steroid treatment,"watchful waiting" may be appropriate in most of asymptomatic patients. Consult an expert to determine if other indications to treat are present. Yes to any Treat patients with AIP who are asymptomatic but have a persistent pancreatic mass on imaging. B Treat patients with AIP and associated IgG4-related sclerosing cholangitis who are asymptomatic but have persistent liver test abnormalities. B Induction of remission Q Does the patient have contraindications to the use of corticosteroids? Yes Consider performing biliary drainage in patients with obstructive jaundice in order to prevent biliary infection, obtain endoscopic brushings and cytology to differentiate IgG4- related sclerosing cholangitis from biliary malignancy. C Consider not performing biliary stenting, and attempting steroid treatment alone, in patients with mild jaundice and no signs of infection. D No Maintainance therapy Type 2 AIP Q Does the patient have non IgG4-related (type 2) autoimmune pancreatitis? Yes Indications for treatment Symptomatic patients Q Is the patient symptomatic related to pancreatic involvement (e.g. obstructive jaundice, abdominal pain, back pain)? Q Is the patient symptomatic related to involvement of other organs (e.g. jaundice due to bile duct stricture)? Yes to any No to all Indications for treatment Asymptomatic patients Q Does the patient have a persistent pancreatic mass on imaging? Q Does the patient have IgG4-related sclerosing cholangitis and persistent liver test abnormalities? Yes to any No to all Treat patients with AIP who are asymptomatic but have a persistent pancreatic mass on imaging. B Treat patients with AIP and associated IgG4-related sclerosing cholangitis who are asymptomatic but have persistent liver test abnormalities. B Induction of remission Q Does the patient have contraindications to the use of corticosteroids? Yes No Initiate corticosteroids as first-line therapy for active AIP in patients who do not have contraindications to their use. A Corticosteroid dosing Initiate prednisone at a dose of 0.6-1.0 mg/kg/day. A Consider tapering prednisone by 5-10 mg/day every 1-2 weeks until a daily dosage of 20 mg, followed by tapering by 5 mg every 2 weeks. C Patients with obstructive jaundice Q Does the patient have obstructive jaundice? Yes No Maintainance therapy Type 2 AIP Q Does the patient have non IgG4-related (type 2) autoimmune pancreatitis? Yes Avoid maintenance therapy in patients with with type 2 AIP. D No Management of relapse Q Does the patient have evidence of disease relapse on follow-up? Yes Consider steroid and steroid-sparing agents (such as immune-modulators or rituximab) for treatment of relapsing AIP. C Consider surgical treatment in selected patients who are refractory to medical therapy. C No Continue management and follow-up as clinically appropriate.