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Transgenic Res 2021 Jun 11. Epub 2021 Jun 11.

Department of Medical Biochemistry and Biophysics, Karolinska Institutet, 171 77, Stockholm, Sweden.

Chronic cholestatic liver diseases including primary sclerosing cholangitis (PSC) present a complex spectrum with regards to the cause, age of manifestation and histopathological features. Current treatment options are severely limited primarily due to a paucity of model systems mirroring the disease. Here, we describe the Keratin 5 (K5)-Cre; Klf5 mouse that spontaneously develops severe liver disease during the postnatal period with features resembling PSC including a prominent ductular reaction, fibrotic obliteration of the bile ducts and secondary degeneration/necrosis of liver parenchyma. Read More

Dig Liver Dis 2021 Jun 8. Epub 2021 Jun 8.

Department of Gastroenterology, Helsinki University Hospital and University of Helsinki, Helsinki, Finland.

Introduction: Risk of post-ERC pancreatitis (PEP) in patients with primary sclerosing cholangitis (PSC) is 1-7.8%. PSC is often associated with inflammatory bowel disease and autoimmune hepatitis, which are usually treated with thiopurines. Read More

Medicine (Baltimore) 2021 Jun;100(23):e25774

Division of Medical Oncology, Department of Medicine, Showa University School of Medicine.

Introduction: Immune checkpoint inhibitors (ICIs), particularly anti-PD-1 antibody, have dramatically changed cancer treatment; however, fatal immune-related adverse events (irAEs) can develop. Here, we describe a severe case of sclerosing cholangitis-like irAE. We report the use of 3 immunosuppressive agents that resulted in the death of the patient due to treatment inefficacy. Read More

Pak J Med Sci 2021 May-Jun;37(3):680-683

Dr. Usman Iqbal Aujla, Consultant Gastroenterologist , Pakistan Kidney and Liver Institute & Research Centre, Lahore, Pakistan.

Background And Objectives: Chronic liver disease (CLD) in children present a broad spectrum of symptoms. Limited resources in Paediatric Hepatology in developing countries like Pakistan present considerable challenges in investigating and treating children with chronic liver disease in a timely fashion. This study aimed to determine the spectrum and outcomes of CLD other than chronic hep B & C virus (HBV& HCV) liver disease in children. Read More

Endoscopy 2021 Jun 8. Epub 2021 Jun 8.

Department and Outpatients' Department of Internal Medicine I, University Hospital Regensburg, Germany.

Clin J Gastroenterol 2021 Jun 8. Epub 2021 Jun 8.

Department of Gastroenterology and Hepatology, Graduate School of Medicine, Kyoto University, 54 Kawahara-cho, Syogoin, Sakyo-ku, Kyoto, 606-8507, Japan.

An 18-year-old woman was admitted to our hospital with fever, diarrhea and painful skin ulcers in both pretibial areas starting 19 days earlier. The skin lesions appeared deep necrotic ulcers with violaceous undermined borders. She had been diagnosed as ulcerative colitis and primary sclerosing cholangitis (PSC) 6 and 5 years before, respectively, and had stopped having regular check-up and refused medication for years. Read More

BMJ Case Rep 2021 Jun 4;14(6). Epub 2021 Jun 4.

Department of Gastroenterology, Kanazawa Municipal Hospital, Kanazawa, Ishikawa, Japan.

A 57-year-old man with lung cancer, previously treated with the programmed death-1 inhibitor pembrolizumab, was evaluated for liver injury and acute pancreatitis. Serum IgG4 levels were not elevated. Contrast-enhanced CT showed pancreatic swelling, contrast unevenness in the liver and thickening of the common bile duct and gall bladder. Read More

Clin Case Rep 2021 May 24;9(5):e04068. Epub 2021 May 24.

Clinic for Gastroenterology, Hepatology and Infectious Diseases Heinrich-Heine-University Düsseldorf Düsseldorf Germany.

This case of secondary sclerosing cholangitis (SSC-CIP) emphasizes the need to provide follow-up care for patients that have recovered from COVID-19 in order to understand the complexity of SARS-CoV-2 associated sequela. Read More

BMC Rheumatol 2021 Jun 3;5(1):16. Epub 2021 Jun 3.

Division of Rheumatology, Department of Pediatrics, University of British Columbia, Vancouver, Canada.

Background: Chronic Recurrent Multifocal Osteomyelitis (CRMO) is a condition characterized by sterile bone inflammation, usually occurring in childhood. Although the etiology remains unclear, this condition has been associated with inflammatory bowel disease (IBD). Primary sclerosing cholangitis (PSC) and Autoimmune Hepatitis (AIH) are also uncommon pediatric conditions with a known association with IBD. Read More

Pediatr Neonatol 2021 May 11. Epub 2021 May 11.

Department of Pediatrics, National Taiwan University Children's Hospital, National Taiwan University, Taipei, Taiwan. Electronic address:

Background: Primary sclerosing cholangitis (PSC) is often associated with ulcerative colitis (UC). We investigated the clinical characteristics of pediatric UC patients with and without PSC.

Methods: We retrospectively recruited children with UC, with and without PSC, from 2006 to 2017 in a tertiary center in Taiwan. Read More

Vnitr Lek 2021 ;67(2):92-96

Inflammatory bowel disease (IBD) includes Crohns disease (CD) and ulcerative colitis (UC). Those are chronic gastrointestinal disorders of inflammatory nature and not fully known etiology. As a result of their immune-mediated mechanism and complex impact on the whole organism other organs than gastrointestinal system may be affected in many ways. Read More

Vnitr Lek 2021 ;67(2):76-83

IgG4-related disease is a recently defined clinical entity that can manifest itself in any organ. The most common gastrointestinal manifestations are diseases of the pancreas (autoimmune pancreatitis type 1) and biliary tree (IgG4-associated cholangitis); involvement of liver parenchyma is uncommon and the affection of tubular organs is very rare. IgG4-related pancreatitis and cholangitis can mimic malignancies in their clinical presentation. Read More

Cells 2021 May 5;10(5). Epub 2021 May 5.

Department of Immunology, Ophthalmology & ENT, Complutense University School of Medicine, 28040 Madrid, Spain.

Cholestatic liver diseases including primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are associated with active hepatic fibrogenesis, which can ultimately lead to the development of cirrhosis. However, the exact relationship between the development of liver fibrosis and the progression of cholestatic liver disease remains elusive. Periductular fibroblasts located around the bile ducts seem biologically different from hepatic stellate cells (HSCs). Read More

Arab J Gastroenterol 2021 May 28. Epub 2021 May 28.

Paediatric Gastroenterology Unit, Department of Paediatrics, King Abdulaziz University Hospital, Jeddah, Saudi Arabia.

Background And Study Aims: Autoimmune liver diseases (ALDs) are a clinico-pathologic spectrum of disorders that share some similarities. They are formally classified as autoimmune hepatitis (AIH), isolated autoimmune sclerosing cholangitis (ASC), and the overlap syndrome of these. We describe the clinical, biochemical, and outcomes data of a cohort of autoimmune ALDs patients in a tertiary care centre. Read More

Hepatology 2021 May 31. Epub 2021 May 31.

USC Research Center for Liver Diseases, Keck School of Medicine, University of Southern California, Los Angeles California, USA.

Autoimmune liver diseases are attributed to a complex interplay of biologic, acquired, and environmental factors. Increased prevalence, later stage of presentation, worse response to standard therapy, and transplant-related disparities have all been reported in racial and ethnic minorities such as Black and Latinx patients with autoimmune liver diseases. While biology and inherited genetic predispositions may partly explain these disparities, definitive and universal genetic variations underlying these differences in outcomes have not been defined. Read More

Am J Case Rep 2021 May 30;22:e930729. Epub 2021 May 30.

Department of Gastroenterology, Gastrointestinal Oncology, and Endocrinology, University Medical Center Goettingen, Goettingen, Germany.

BACKGROUND Immunoglobulin (Ig) G4-related disease is a rare disease of unknown pathophysiology, which can affect multiple organs leading to tissue fibrosis and organ failure. The present case report describes a patient with systemic IgG4-related disease (IgG4-RD) that occurred over a 1-year period and affected multiple organs at different times. Imaging studies, interventional procedures, changes in laboratory parameters, and histopathology demonstrate the novel and known aspects of this disease before and during prednisolone monotherapy and in combination with azathioprine. Read More

Gastroenterol Hepatol 2021 May 27. Epub 2021 May 27.

Departamento de Gastroenterología, Hepatología y Unidad de Trasplante Hepático, Hospital Universitari y Politècnic La Fe, 46026 Valencia, España; Instituto de Investigación Sanitaria La Fe, 46026 Valencia, España; Centro de Investigación Biomédica en Red del Área de Enfermedades Hepáticas y Digestivas (CIBEREHD) del Instituto de Salud Carlos III de Madrid, 28029 Madrid, España; Departamento de Medicina, Universitat de València, 46010 Valencia, España. Electronic address:

Background And Aims: Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease that typically affects middle-aged men with ulcerative colitis (UC). However, recent studies point out to epidemiological changes. Our aim was to determine if the epidemiology, clinical course and outcome of patients with PSC followed at a reference hepatology center resemble what is described in the literature. Read More

Liver Int 2021 May 29. Epub 2021 May 29.

Sheila Sherlock Liver Centre, Royal Free London NHS Foundation Trust and UCL Institute for Liver and Digestive Health, University College of London, London, United Kingdom.

Background & Aims: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease in need of accurate biomarkers for stratification and as surrogates for clinical endpoints in trials. Quantitative liver fibrosis assessment by collagen proportionate area (CPA) measurement has been demonstrated to correlate with clinical outcomes in chronic hepatitis C, alcohol-related and non-alcoholic fatty liver disease. We aimed to investigate the ability of CPA to quantify liver fibrosis and predict clinical events in PSC. Read More

BMJ Case Rep 2021 May 28;14(5). Epub 2021 May 28.

Gastroenterology, NHS Greater Glasgow and Clyde, Glasgow, UK.

A 69-year-old retired miner with stage 4 non-small-cell lung cancer presented with a 2-month history of obstructive liver function tests following nivolumab immunotherapy. His case had not responded to high dose prednisolone or mycophenolate and he was admitted for investigation. MR cholangiopancreatography demonstrated areas of intrahepatic biliary tree beading and stricturing, in keeping with sclerosing cholangitis. Read More

Transplantation 2021 May 25. Epub 2021 May 25.

Multi Organ Transplant Program, University Health Network, Toronto, ON, Canada. Blood and Marrow Transplant Program, The Ottawa Hospital, Ottawa, ON, Canada. Department of Pathology, University of Pittsburgh, Pittsburgh, PA. Department of Medical Imaging, University Health Network, Toronto, ON, Canada. Ottawa Stem Cell Program, Canadian Blood Services, Ottawa, ON, Canada.

Background: Primary sclerosing cholangitis (PSC) is an indication for liver transplantation, but recurrence after liver transplantation is associated with poor outcomes often requiring repeat transplantation. We investigated whether autologous hematopoietic stem cell transplantation (aHSCT) could be used to stop progression of recurrent PSC and promote operational tolerance.

Methods: Twelve patients with recurrent PSC were fully evaluated and 5 were selected for aHSCT. Read More

Invest New Drugs 2021 May 28. Epub 2021 May 28.

Division of Thoracic Oncology, Shizuoka Cancer Center, 1007 Shimonagakubo, Nagaizumi, Sunto-gun, Shizuoka, 411-8777, Japan.

Background Immune-related hepatotoxicity is often regarded as immune-related hepatitis (irHepatitis) despite including immune-related sclerosing cholangitis (irSC). This study examined the clinical differences between irSC and irHepatitis. Methods A single-center retrospective study of 530 consecutive patients who received immunotherapy between August 2014 and April 2020 was performed. Read More

Cureus 2021 Apr 23;13(4):e14648. Epub 2021 Apr 23.

Internal Medicine/Infectious Disease, University of Michigan, Ann Arbor, USA.

species are gram-negative, non-lactose fermenting, aerobic bacilli closely related to genus. () is an emergent human pathogen that is difficult to differentiate from other species by conventional methods. It is known to infect immunocompromised hosts, has the propensity for abscess formation, and is known for its multidrug resistance. Read More

Inflamm Bowel Dis 2021 May 27. Epub 2021 May 27.

Department of Gastroenterology, Shengjing Hospital of China Medical University, Shenyang, Liaoning, China.

JHEP Rep 2021 Jun 30;3(3):100286. Epub 2021 Mar 30.

Department of Medicine - DIMED, University of Padova, Padua, Italy.

Background & Aims: Primary sclerosing cholangitis (PSC) is a rare cholangiopathy of unknown aetiopathogenesis. The aim of this study was to evaluate cellular senescence (CS) marker expression in cholangiocytes of patients with PSC and their correlation with clinical-pathological features and prognosis.

Methods: Thirty-five patients with PSC with at least 1 available liver sampling were included. Read More

JHEP Rep 2021 Jun 31;3(3):100282. Epub 2021 Mar 31.

Department of Internal Medicine, Division of Gastroenterology and Hepatology, Landspitali-National University Hospital of Iceland, Reykjavik, Iceland.

Background & Aims: The incidence of cirrhosis in Iceland has been the lowest in the world with only 3 cases per 100,000 inhabitants. Alcohol consumption has almost doubled in Iceland from 1980 to 2016. Obesity has also risen and hepatitis C virus has spread among people who inject drugs in Iceland. Read More

World J Gastroenterol 2021 May;27(19):2257-2269

Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Osaka-Sayama 589-8511, Japan.

Solitary organ autoimmune disorders, formerly known as autoimmune pancreatitis (AIP), autoimmune sialadenitis, and autoimmune sclerosing cholangitis, are now considered organ-specific manifestations of systemic immunoglobulin G4-related disease (IgG4-RD). AIP and IgG4-RD are characterized by elevated serum concentration of IgG4 antibody (Ab), accumulation of IgG4-expressing plasmacytes in the affected organs, and involvement of multiple organs. It is well established that enhanced IgG4 Ab responses are a hallmark of AIP and IgG4-RD for diagnosis and monitoring disease activity. Read More

Gut 2021 May 25. Epub 2021 May 25.

Division of Gastroenterology and Hepatology, Key Laboratory of Gastroenterology and Hepatology, Ministry of Health, State Key Laboratory for Oncogenes and Related Genes, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai Institute of Digestive Disease, Shanghai, China

Objective: Multiple clinical similarities exist between IgG4-related sclerosing cholangitis (IgG4-SC) and primary sclerosing cholangitis (PSC), and while gut dysbiosis has been extensively studied in PSC, the role of the gut microbiota in IgG4-SC remains unknown. Herein, we aimed to evaluate alterations of the gut microbiome and metabolome in IgG4-SC and PSC.

Design: We performed 16S rRNA gene amplicon sequencing of faecal samples from 135 subjects with IgG4-SC (n=34), PSC (n=37) and healthy controls (n=64). Read More

Transplantation 2021 May 25. Epub 2021 May 25.

Center for Liver Disease and Transplantation, Department of Surgery, Columbia University Irving Medical Center, New York, NY. Department of Pediatrics, Columbia University Irving Medical Center, New York, NY.

Transpl Int 2021 May 24. Epub 2021 May 24.

Erasmus MC Transplant Institute, Department of Gastroenterology and Hepatology, Erasmus MC University Medical Center Rotterdam, Rotterdam, The Netherlands.

Background: Liver transplantation for primary sclerosing cholangitis (PSC) can be complicated by recurrence of PSC (rPSC). This may compromise graft survival but the effect on patient survival is less clear. We investigated the effect of post-transplant rPSC on graft and patient survival in a large European cohort. Read More

Gastroenterol Hepatol 2021 May 20. Epub 2021 May 20.

Alfa Institute of Gastroenterology - Hospital das Clínicas da Universidade Federal de Minas Gerais, Brazil.

Background And Aims: Inflammatory bowel diseases (IBD) are chronic conditions that may be accompanied by autoimmune liver disease (AILD), most commonly primary sclerosing cholangitis (PSC). The objective of this study was to evaluate the behaviour of patients with IBD associated with AILD and compare a PSC group with a non-PSC group.

Methods: Medical records of patients with IBD associated with PSC, autoimmune cholangitis, primary biliary cholangitis, small-duct PSC, autoimmune hepatitis (AIH) and overlapping syndromes were assessed. Read More