Eosinophilic Cholangitis: A Self-Limited Cause of Extrahepatic Biliary Obstruction

The first case of eosinophilic cholecystitis with radiographically documented biliary tract obstruction is presented. Differences between eosinophilic cholecystitis with and without obstruction in terms of gender predilection and the incidence of associated cholelithiasis suggest these may be two distinct disease processes. The dense eosinophilic infiltration of the porta hepatis seen in the present case has led us to believe that extrahepatic obstruction was due to "eosinophilic cholangitis." The self-limited nature of eosinophilic cholangitis makes it an essential consideration in the evaluation of presumed neoplasms of the porta hepatis.     

PBC应命名为原发性胆汁性胆管炎(Primary Biliary Cholangitis)

自1950年Ahrens EH Jr首次将PBC命名为原发性胆汁性肝硬化（Primary Biliary Cirrhosis）后,该诊断一直沿用至今。我们在临床实践中发现原发性胆汁性肝硬化这一诊断不能准确反映出大部分具有中小胆管慢性、非化脓性、毁损性炎症患者的临床和病理特征。Kuntz E教授在他主编的新版国际肝病领域权威教科书中提出了原发性胆汁性肝硬化（Primary Biliary Cirrhosis）应改名为原发性胆汁性胆管炎（Primary Biliary Cholangitis）,两者的缩写都是PBC。这一疾病名称的改变代表对这类疾病的进一步认识,对其定位十分确切。     

UK-Wide Multicenter Evaluation of Second-line Therapies in Primary Biliary Cholangitis

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Latest and Emerging Therapies for Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis

Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are the two most common causes of chronic cholestatic liver disease in adults. In PBC, therapy with ursodeoxycholic acid (UDCA) is safe and has been associated with tangible biochemical, histologic, and survival benefits. However, a need for different or adjuvant therapies remains for specific subsets of PBC patients, including those who do not respond to UDCA and those who have advanced histologic disease at presentation. Similarly, beneficial therapies for disease-related symptoms that do not typically respond to UDCA (eg, fatigue and pruritus) are still needed. In contrast to PBC, no medical therapy of proven benefit has been identified for patients with PSC. In PBC and PSC, adequate management of complications of chronic cholestasis is important. For both diseases, liver transplantation is the only curative option.     

Predictive Model of Ursodeoxycholic Acid Treatment Response in Primary Biliary Cholangitis

Background and AimsAlthough ursodeoxycholic acid (UDCA) treatment in primary biliary cholangitis is effective in many patients, there are still many people who respond poorly to it. Identifying and intervening these patients early is important. Therefore, exploring the risk factors and proposing a predictor index to predict the UDCA treatment nonresponse earlier among primary biliary cholangitis patients were the aims of this research.MethodsA total of 135 primary biliary cholangitis patients treated with UDCA (13–15 mg/kg/d) were enrolled in this retrospective study. The response to treatment was evaluated based on Paris I criteria. The univariate and logistic multivariate regression analyses were adopted to determine the independent risk factors and propose a predictor index. Receiver operating characteristic curve was used to evaluate the predictive ability of the predictor index.ResultsTotal bilirubin, albumin, globulin, immunoglobin M, and aspartate aminotransferase-to-platelet ratio index were the five independent risk factors associating with early biochemical nonresponse to UDCA treatment. Based on these factors, we established a predictor index with the predictive value being 0.886 (sensitivity: 82.80%, specificity: 84.40%).ConclusionsWe developed a predictor index that had an accurate prediction of the early biochemical nonresponse to UDCA treatment, which is expected to provide valuable information for the high-risk group before treatment begins.