Guide
Autoimmune pancreatitis (AIP) is a chronic, hormone-reactive inflammatory disease of pancreatic fiber, often clinically presenting as obstructive jaundice, with or without pancreatic parenchymal mass, histologically lymphoplasmic infiltrates and chronic fibrosis, and therapeutically showing a high sensitivity to hormonal drugs.
This article mainly reviews the diagnosis and treatment of AIP to help clinicians better understand the disease.
Typing and diagnosis of AIP: type 1 and type 2
The international consensus of AIP diagnostic criteria divides AIP into types 1 and 2 based on histopathological features.
Type 1: i.e., lymphoplastic sclerosing pancreatitis (LPSP), associated with serum immunoglobulin G4 (IgG4), usually with a characteristic elevation of serum IgG4 levels and other IgG4-related disorders. At present, Asian patients are mainly type 1, accounting for more than 96%.
Type 2: idiopathic ductal central pancreatitis (IDCP), associated with epithelial granulocyte damage, may be accompanied by ulcerative colitis. Type 2 AIP in Europe and the United States is not uncommon, showing a mixed prevalence of type 1 (80%).
The international standard describes the typical diagnostic characteristics of AIP from six aspects: pancreatic parenchymal imaging, main pancreatic duct imaging, serology and extrapancreatic lesions, histology, and hormone therapy response, and divides the evidence intensity into grades 1 and 2 according to evidence-based medicine, as the basis for the current clinical diagnostic work, see Table 1.
Table 1 Type 1 AIP International Diagnostic Standards
A diagram summary: The treatment of AIP
Treatment for AIP is shown in Figure 1, but close follow-up and monitoring of the likelihood of underlying malignancy is required.
Figure 1 Treatment of AIP
IgG4-SC, IgG4-associated sclerosing cholangitis; MMF, mycophenolate mofetil; 6-MP, 6-mercaptopurine; AZA, azathioprine
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