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Autoimmune pancreatitis

Background

Overview

Definition
AIP is an IgG4 systemic disease characterized by obstructive jaundice, abdominal pain, and acute pancreatitis.
1
Pathophysiology
The exact cause of AIP is unknown; however, autoimmune cause (association with HLA haplotype) is postulated.
2
Disease course
Th1 type of immune response is predominantly involved in the development of AIP, which presents as type 1 and type 2 subtypes with symptoms of obstructive jaundice, acute pancreatitis, abdominal pain, and IBD. Manifestations of other organ involvement include biliary disease, symptoms of Sjogren's disease (due to salivary gland involvement), lung nodules, interstitial nephritis, and retroperitoneal fibrosis.
3
Prognosis and risk of recurrence
The risk of mortality increased with OR 2.07 (p = 0.02; 95% CI 1.07-3.55) in type 1 AIP.
4

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of autoimmune pancreatitis are prepared by our editorial team based on guidelines from the European Pancreatic Club (EPC/IAP 2017). ...
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Classification and risk stratification

Predictors of relapse: as per EPC/IAP 2017 guidelines, predictors of relapse include:
remarkably high serum IgG4 levels, such as > 4 times the ULN before treatment
persistently high serum IgG4 levels after steroid treatment
diffuse enlargement of the pancreas
proximal type of IgG4-SC
extensive multi-organ involvement (≥ 2 other organs involved).
B
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Medical management

Indications for treatment, symptomatic patients: as per EPC/IAP 2017 guidelines, 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

More topics in this section

  • Indications for treatment (asymptomatic patients)

  • Induction of remission

  • Corticosteroid dosing

  • Maintenance therapy (type 1 AIP)

  • Maintenance therapy (type 2 AIP)

  • Management of relapse

Specific circumstances

Patients with obstructive jaundice
As per EPC/IAP 2017 guidelines:
Consider performing biliary drainage in patients with obstructive jaundice in order to prevent biliary infection, obtain endoscopic brushings and cytology to differentiate IgG4-SC 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