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- W2997281835 abstract "Background: Immunoglobulin G4-associated cholangitis (IAC) is a major manifestation of IgG4-related disease (IgG4-RD), a multi-organ inflammatory disorder. Since the disease mimics malignancies of the pancreatobiliary tract, misdiagnosis and unnecessary surgery are common. Methods: All patients resected for presumed PHC at our institution between 1984 and 2015 were included. Benign histological specimens were re-evaluated by a pathologist and scored according to the international pathological consensus criteria for IgG4-RD. Patients with benign disease postoperative who were still alive, were re-evaluated to assess IgG4 serum levels and IgG4/IgG RNA ratio in order to detect persistent activity of IgG4-RD. Results: 321 patients underwent liver and bile duct resection for presumed PHC. Of all patients 15% (47/321) were found to have benign disease on histological examination. 45% (21/47) of patients with benign disease had evidence of IAC based on histological criteria (n=17) or laboratory parameters (n=4). The remaining specimens showed unclassified sclerosing inflammation. Out of 15 patients with IAC who were still alive and were re-evaluated postoperatively, 8 had ongoing active disease. Conclusion: Benign biliary disorders mimicking PHC have led to a considerable number of liver and bile duct resections. There was evidence of IAC in 45% of the patients. When left untreated, IgG4-RD can remain active for years. More awareness and novel diagnostic tests might reduce misdiagnosis and unnecessary surgery." @default.
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- W2997281835 date "2019-01-01" @default.
- W2997281835 modified "2023-10-16" @default.
- W2997281835 title "IgG4-associated cholangitis mimicking perihilar cholangiocarcinoma; a persistent dilemma" @default.
- W2997281835 doi "https://doi.org/10.1016/j.hpb.2019.10.1392" @default.
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