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    • Autoimmune Pancreatitis
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    • Clinical characteristics of immunoglobulin IgG4-related sclerosing cholangitis: Comparison of cases with and without autoimmune pancreatitis in a large cohort: Influence Statistics

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      Concepts for whichthey havehas direct influence:Autoimmune pancreatitis,Autoimmune pancreatitis aip,Sclerosing cholangitis,Igg4 sclerosing,Cholangitis igg4,Bile duct,Igg4 autoimmune,Wall thickening.

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      Clinical characteristics of immunoglobulin IgG4-related sclerosing cholangitis: Comparison of cases with and without autoimmune pancreatitis in a large cohort

      Abstract

      BACKGROUND: The clinical characteristics of IgG4-related sclerosing cholangitis (IgG4-SC) especially without autoimmune pancreatitis (AIP) have not been investigated in a large cohort.

      AIMS: To clarify the clinical characteristics of IgG4-SC and IgG4-SC without AIP.

      METHODS: We retrospectively reviewed imaging, serology, other organ involvement (OOI) and histology of 872 patients with IgG4-SC who participated in a Japanese nationwide survey in 2019, and compared these items between IgG4-SC with and without AIP.

      RESULTS: AIP was present in 83.7% (730/872) of IgG4-SC. In IgG4-SC, bile duct wall thickening was observed on ultrasound (528/650; 81.2%), computed tomography (375/525; 71.4%) and magnetic resonance imaging or cholangiopancreatography (290/440; 65.9%). An elevated serum IgG4 level (≥ 135 mg/dL) was found in 88.0% (322/366). IgG4-related OOI other than AIP was observed in 25.2% (211/836). The proportion of females was significantly higher in IgG4-SC without AIP (28.9% vs. 20.1%; p = 0.025). Hilar stricture was the most common cholangiographic type in IgG4-SC without AIP (39/107; 36.4%).There were no significant differences between IgG4-SC with and without AIP in the rates of bile duct wall thickening, elevated serum IgG4 level, or IgG4-related OOI.

      CONCLUSIONS: The clinical characteristics of IgG4-SC was similar between IgG4-SC with and without AIP in a large cohort.

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