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sclerosing cholangitis in critically ill patients： an important and easily ignored problem based on a German experience

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Frontiers of Medicine 2014年第1期8卷 118-126页

作者： Ting Lin Kai Qu Xinsen Xu Min Tian Jie Gao Chun Zhang Ying Di Yuelang Zhang Chang Liu Department of Hepatobiliary Surgery Surgical Intensive Care Unit Department of Imaging the First Affiliated Hospital School ofMedicine Xi 'an Jiaotong University Xi 'an 710061 China

Intensive care unit （ICU） is important in the rehabilitation of critically ill patients. In the past decades, many patients who received aggressive treatment in ICU developed sclerosing cholangitis in multiple centers. sclerosing cholangitis in critically ill patients （SC-CIP） is a relatively new issue. To investigate the causes, clinical manifestation, treatment, and prognosis of SC-CIP, we searched for published cases in the databases of PubMed, Highwire, and Elsevier from 2001 to 2012. Data were extracted using a standard form and retrospectively analyzed. Twelve eligible studies covering 88 patients, with 64 men and 24 women, were mainly reported by German centers. The mean age was 49.8 years. All of the patients recovered from critical illnesses, such as trauma, infection, burn, and major surgeries. High pressure positive end-expiratory pressure （PEEP, peak level at 12.8 cm H20） was utilized for all patients, with the average duration of 36.3 d. In addition, vasopressor agents were administered in approximately 60% of SC-CIE A rapid increase in cholestasis and irregular strictures in the intrahepatic bile ducts was observed in the following months. With an average follow-up period of 17.9 months, poor outcomes were observed in 54 patients, including 34 deaths. In conclusion, ischemic injury of the biliary tree, which may be affected by PEEP and/or vasopressor administration, affects cholangiopathic procedure. As a newly discovered type of secondary sclerosing cholangitis, SC-CIP is a severe progressive complication of patients in ICU and should be carefully monitored by clinicians.

关键词： intensive care unit sclerosing cholangitis ischemic injury prognosis systemic review

Healthy Donor Fecal Microbiota Transplantation for Recurrent Bacterial cholangitis in Primary sclerosing cholangitis-A Single Case Report

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Journal of Clinical and Translational Hepatology 2018年第4期6卷 438-441页

作者： Cyriac Abby Philips Philip Augustine Nikhil Phadke The Liver Unit Cochin Gastroenterology GroupP.A.A.Ernakulam Medical CentreNational Highway Bypass(NH-66)PalarivattomKochiKeralaIndia Gastroenterology Cochin Gastroenterology GroupP.A.A.Ernakulam Medical CentreNational Highway Bypass(NH-66)PalarivattomKochiKeralaIndia Molecular Cellular and Developmental BiologyGenepath DxPhadke HospitalShivajinagarPuneMaharashtraIndia

Recurrent acute bacterial cholangitis is a unique indication for liver transplantation in primary sclerosing cholangitis.We present the first report on utility of healthy donor fecal transplantation for management of recurrent acute bacterial cholangitis in a primary sclerosing cholangitis patient.We demonstrate the striking liver biochemistry,bile acid and bacterial community changes following intestinal microbiota transplantation associated with amelioration of recurrent cholangitis.

关键词： FMT sclerosing cholangitis Stool transplant Gut microbiome Microbiota

Cholangiocarcinoma, Primary sclerosing cholangitis, or IgG4-sclerosing cholangitis: Similar Presentations with Different Managements

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Case Reports in Clinical Medicine 2023年第4期12卷 108-112页

作者： Anas Mahmoud Abdalla Mohamed Nizar Alyassin Matthew Grossman Yana Cavanagh St. Joseph’s University Medical Center Paterson USA

In our case, we present a case of an 80-year-old male who was referred to the gastroenterologist for evaluation of a suspicious mass. CT imaging at the time had shown intrahepatic and extrahepatic biliary dilations, and the patient was thought to have a pancreatic or a common bile duct mass. A mass biopsy showed no malignancy, and further evaluation was warranted. The patient was found to have elevated IgG4 levels and was diagnosed with IgG4-sclerosing cholangitis (IgG4-SC). IgG4 has been found to create a wide array of pathologies, including autoimmune pancreatitis, dacryoadenitis, and sialadenitis. These pathologies have been grouped under an IgG4-Related Disease (IgG4-RD) category. In some cases, this IgG4-RD can present as a subclass of primary sclerosing cholangitis due to immune depositions and swelling of the CBD. Due to the strictures caused by the sclerosing cholangitis, intrahepatic and extrahepatic dilations might be found on endoscopic ultrasound (EUS). It is imperative to differentiate this from a malignant mass as the early recognition and treatment of IgG4-SC can lead to complete resolution. In this case report, we present a case of a patient who was found to have IgG4-SC and responded well to steroid treatment.

关键词： Cholangiocarcinoma sclerosing cholangitis IgG4 Related Disease IgG4 IgGsclerosing cholangitis ERCP CBD Dilation

Diagnosis of Liver Involvement in Primary Sj(o)gren Syndrome

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Journal of Clinical and Translational Hepatology 2013年第2期1卷 94-102页

作者： Pilar Brito Zeron Soledad Retamozo Albert Bové Belchin Adriyanov Kostov Antoni Sisó Manuel Ramos-Casals Sj(o)gren Syndrome Research Group(AGAUR) Laboratory of Autoimmune Diseases Josep Font Institut d'Investigacions Biomèdiques August Pi i Sunyer(IDIBAPS) Department of Autoimmune Diseases ICMiD Hospital Clinic Barcelona Spain Primary Care Research Group IDIBAPS Centre d'Assistència_Primària ABS Les Corts GESCLINIC Barcelona Spain

Liver involvement was one of the first extraglandular manifestations to be reported in patients with primary Sj(o)gren syndrome (SS).In the 1990s,a study of liver involvement in patients with primary SS integrated the evaluation of clinical signs of liver disease,liver function and a complete panel of autoantibodies.Recent developments in the field of hepatic and viral diseases have significantly changed the diagnostic approach to liver involvement in SS.The most recent studies have shown that,after eliminating hepatotoxic drugs and fatty liver disease,the two main causes of liver disease in primary SS are chronic viral infections and autoimmune liver diseases.The differential diagnosis of liver disease in primary SS (viral vs autoimmune) is clinically important,since the two processes require different therapeutic approaches and have different prognoses.With respect to viral infections,chronic HCV infection is the main cause of liver involvement in SS patients from the Mediterranean area,while chronic HBV infection may be the main cause of liver involvement in SS patients from Asian countries.After eliminating viral hepatitis,primary biliary cirrhosis (PBC) should be considered the main cause of liver disease in primary SS.PBC-related SS patients may have a broad spectrum of abnormalities of the liver,including having no clinical or analytical data suggestive of liver disease.Autoimmune hepatitis (AIH) is the second most frequently found autoimmune liver disease to be associated with SS (all reported cases are type I),and nearly 10% of these patients have an AIH-PBC overlap.Finally,IgG4-related disease must be investigated in patients with SS presenting with sclerosing cholangitis,especially when autoimmune pancreatitis or retroperitoneal fibrosis are also present.

关键词： Sj(o)gren syndrome Liver disease Hepatitis B virus Hepatitis C virus Primary biliary cirrhosis Autoimmune hepatitis sclerosing cholangitis