Publication details
Autoimmune Pancreatitis - Recent Advances
| Authors | |
|---|---|
| Year of publication | 2010 |
| Type | Article in Periodical |
| Magazine / Source | DIGESTIVE DISEASES |
| MU Faculty or unit | |
| Citation | |
| Field | Other specializations of internal medicine |
| Keywords | Autoimmune pancreatitis. IgG4-related sclerosing disease. Corticosteroid therapy. Granulocyte epithelial lesion |
| Description | Autoimmune pancreatitis (AIP) is recognized as a distinct clinical entity, identified as a chronic inflammatory process of the pancreas in which the autoimmune mechanism is involved. Clinically and histologically, AIP has two subsets: type 1 lymphoplasmatic sclerosing pancreatitis with abundant infiltration of the pancreas and other affected organs with immunoglobulin G4 positive plasma cells, and type 2 duct centric fibrosis, characterized by granulocyte epithelial lesions in the pancreas without systemic involvement. In the diagnosis of AIP, two diagnostic criterions are used the HISORt criteria and Asian Diagnostic Criteria. Typical signs of AIP are concomitant disorders in other organs (kidney, liver, biliary tract, salivary glands, colon, retroperitoneum, prostate). Novel clinicopathological entity was proposed as an IgG4 related sclerosing disease (IgG4 RSC). Extensive IgG4 positive plasma cells and T lymphocyte infiltration is a common characteristics of this disease. Recently, IgG4 RSC syndrome was extended to a new entity, characterized by IgG4 hypergammaglobulinemia and IgG4-positive plasma cell infiltration, this being considered an expression of a lymphoproliferative disease, IgG4-positive multiorgan lymphoproliferative syndrome. This syndrome includes Mikulicz s disease, mediastinal fibrosis, autoimmune hypophysitis, and inflammatory pseudotumor lung, liver, breast. In the therapy of AIP, steroids constitute first-choice treatment. High response to the corticosteroid therapy is an important diagnostic criterion. In the literature, there are no case-control studies that determine if AIP predisposes to pancreatic cancer. Undoubtedly, AIP is currently a hot topic in pancreatology. |