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Autoimmune pancreatitis and IgG4-related sclerosing disease

Abstract

Autoimmune pancreatitis (AIP) is a unique form of pancreatitis in which the pathogenesis is suspected to involve autoimmune mechanisms. AIP sometimes mimics pancreatic cancer in its presentation, but as AIP responds dramatically to steroid therapy, accurate diagnosis is necessary. AIP is currently diagnosed on the basis of a combination of characteristic clinical, serological, morphological and histopathological features. However, its diagnosis remains a clinical challenge and there are no internationally agreed diagnostic criteria. Another type of AIP called 'idiopathic duct-centric chronic pancreatitis' or 'AIP with granulocytic epithelial lesion' has been reported in Western countries. IgG4-related sclerosing disease is a systemic disease in which IgG4-positive plasma cells and T lymphocytes extensively infiltrate various organs. Organs with tissue fibrosis and obliterative phlebitis, such as the pancreas, salivary gland and retroperitoneum, show clinical manifestations; AIP seems to represent one manifestation of IgG4-related sclerosing disease. As a mass is formed in most cases of IgG4-related sclerosing disease, a malignant tumor is frequently suspected on initial presentation. Clinicians should consider IgG4-related sclerosing disease in the differential diagnosis to avoid unnecessary surgery.

Key Points

  • Autoimmune pancreatitis (AIP) should be diagnosed on the basis of a combination of characteristic clinical, serological, morphological and histopathological features

  • Given than AIP responds dramatically to steroid therapy, accurate differentiation from pancreatic cancer is important

  • IgG4-related sclerosing disease is a systemic disease, in which IgG4-positive plasma cells and T lymphocytes extensively infiltrate various organs

  • AIP may be one manifestation of IgG4-related sclerosing disease

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Figure 1: Radiological findings of patients with autoimmune pancreatitis (AIP).
Figure 2: Histological findings of the pancreas of patients with autoimmune pancreatitis.
Figure 3: Standard treatment strategy for autoimmune pancreatitis.
Figure 4: Schematic illustration of the concept of IgG4-related sclerosing disease, which is a systemic disease in which IgG4-positive plasma cells and T lymphocytes extensively infiltrate various organs.
Figure 5: Histological findings of IgG4-related sclerosing diseases.

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Acknowledgements

This work was partially supported by a grant-in-aid for refractory pancreatic disease from the Ministry of Health, Labor and Welfare of Japan.

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Kamisawa, T., Takuma, K., Egawa, N. et al. Autoimmune pancreatitis and IgG4-related sclerosing disease. Nat Rev Gastroenterol Hepatol 7, 401–409 (2010). https://doi.org/10.1038/nrgastro.2010.81

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