EASL Clinical Practice Guidelines: Management of cholestatic liver diseases

,

doi:10.1016/j.jhep.2009.04.009

Cited by 1,484 publications

(582 citation statements)

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“…The role of endoscopic intervention for asymptomatic DSs (i.e., in the absence of related signs or symptoms) is less clear, although it is not uncommon to see patients undergo multiple ERCs in this clinical scenario. However, given the risk of ERC in PSC (7.3%‐20%)18, 29 and the uncertain benefits (at least in patients without signs or symptoms of DS), additional studies are needed to determine the impact of endoscopic therapy on the natural history and survival in PSC, especially in the absence of biliary obstructive symptoms 30…”

Section: Discussionmentioning

confidence: 99%

Dominant strictures in primary sclerosing cholangitis: A multicenter survey of clinical definitions and practices

Hilscher

Tabibian

Carey

et al. 2018

Hepatology Communications

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Dominant strictures (DSs) of the biliary tree occur in approximately 50% of patients with primary sclerosing cholangitis (PSC) and may cause significant morbidity. Nevertheless, the definition and management of DSs lacks consensus. We aimed to better understand current perceptions and practices regarding PSC‐associated DSs. We conducted an anonymous, 23‐question, survey‐based study wherein electronic surveys were distributed to 131 faculty in the Division of Gastroenterology and Hepatology at the three Mayo Clinic campuses (Rochester, Scottsdale, and Jacksonville) as well as the affiliated practice network. Responses were aggregated and compared, where applicable, to practice guidelines of the American Association for the Study of Liver Diseases and European Association for the Study of the Liver. A total of 54 faculty (41.2%) completed the survey, of whom 24 (44.4%) were hepatologists, 21 (38.9%) gastroenterologists, and 9 (16.7%) advanced endoscopists. One of the major study findings was that there was heterogeneity among participants' definition, evaluation, management, and follow‐up of DSs in PSC. The majority of participant responses were in accordance with societal practice guidelines, although considerable variation was noted. Conclusion: Despite the prevalence and morbidity of DSs in PSC, clinical perceptions and practices vary widely among hepatologists, gastroenterologists, and advanced endoscopists who manage these patients, even within a single health care system. Further studies are needed to address these variations, develop general and evidence‐based consensus, and increase adherence to societal guidelines. (Hepatology Communications 2018;2:836‐844)

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Section: Discussionmentioning

confidence: 99%

Dominant strictures in primary sclerosing cholangitis: A multicenter survey of clinical definitions and practices

Hilscher

Tabibian

Carey

et al. 2018

Hepatology Communications

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“…All patients were recruited in two Polish university Centres (Pomeranian Medical University, Szczecin and Medical University of Warsaw, Warsaw). The diagnosis of PBC was established according to the European Association for the Study of Liver (EASL) criteria [11]. The presence of acute and chronic liver diseases other than PBC, as well as liver tumors, was excluded in all patients.…”

Section: Subjectsmentioning

confidence: 99%

Genetic Risk Factors for Autoimmune Thyroid Disease might Affect the Susceptibility to and Modulate the Progression of Primary Biliary Cholangitis

Kuś

Arłukowicz-Grabowska

Szymański

et al. 2017

JGLD

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Background & Aims: Patients with primary biliary cholangitis (PBC) frequently suffer from extrahepatic autoimmune conditions, of which autoimmune thyroid disease (AITD) is one of the most common. Previous studies identified several genetic variants increasing the odds of developing AITD. Here we investigate whether AITD-associated polymorphisms might also play a role in the development and clinical course of PBC and PBC associated with AITD (PBC-AITD).Methods: To this end, we prospectively recruited 230 patients with PBC and 421 healthy controls. Among recruited patients, 64 (30.9%) had PBC-AITD as diagnosed by elevated serum TPO-antibodies. In all subjects we genotyped 10 variants previously associated with AITD.Results: We detected significant associations between the PTPN22 polymorphism and risk of developing PBC (rs2476601, OR=1.43, P=0.035) as well as PBC-AITD (OR=1.74, P=0.028). The IL2RA polymorphism was associated with liver cirrhosis (rs41295061, OR=1.76, P=0.033) whereas the MMEL1 polymorphism increased the risk of requiring liver transplantation (rs2843403, OR=1.70, P=0.023). Although no significant differences in clinical or biochemical characteristics between patients with PBC and PBC-AITD were seen (all P>0.05), liver function tests and metabolic traits in PBC patients were significantly (all P<0.05) affected by the CTLA4 (rs3087243), MMEL1 (rs2843403), PTPN22 (rs2476601) and RNASET2 (rs9355610) variants.Conclusion: Our study demonstrates the existence of a genetic overlap between PBC and AITD. Apparently, genetic variants known to increase the AITD risk might affect the clinical course of PBC. On the other hand, AITD per se does not seem to significantly influence the natural history of PBC.Abbreviations: AITD: autoimmune thyroid disease; ALP: alkaline phosphatase; ALT: alanine transaminase; AMA: anti-mitochondrial autoantibodies; AST: aspartate aminotransferase; DM1: diabetes mellitus type 1; ELISA: enzyme-linked immunosorbent assay method; GGT: gamma-glutamyl transpeptidase; GD: Graves’ disease; GWAS: genome-wide association studies; HT: Hashimoto thyroiditis; HWE: Hardy-Weinberg equilibrium; Lyp: lymphoid-specific protein tyrosine phosphatase non-receptor type 22; OR: odds ratio; PBC: primary biliary cholangitis; RA: rheumatoid arthritis; SLE: systemic lupus erythematosus; TPOAb: thyroid peroxidase antibody; UDCA: ursodeoxycholic acid.

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“…The next step is testing for serum antimitochondrial antibodies (AMA) and IgG4 levels. The diagnosis of PBC, which represents the major cause of small-duct biliary diseases, can be made with confidence in a patient with high-titer AMA (≥1/40) and elevated alkaline phosphatase activitiy without liver biopsy [5]. IgG4-related disease is a newly recognized fibroinflammatory disease characterized by tumefactive lesions, dense lymphoplasmacytic infiltrates rich in IgG4-positive plasma cells, storiform fibrosis, (often but not always) elevated serum IgG4 concentrations, and excellent response to steroid therapy [6,7].…”

Section: Tablementioning

confidence: 99%

Cholestatic Liver Disease

Jüngst

Lammert²

2013

Dig Dis

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Cholestasis develops as a consequence of impaired bile formation and/or bile flow and can be classified as intra- or extrahepatic. Chronic cholestatic diseases are mostly intrahepatic with the exception of primary and secondary sclerosing cholangitis affecting intra- and extrahepatic bile ducts. Recent genome-wide association studies have confirmed major histocompatibility complex associations and discovered multiple susceptibility loci in primary biliary cirrhosis and primary sclerosing cholangitis, providing new insights into disease pathogenesis, which may translate into more precise therapeutic prevention and intervention in the future. Diagnostic steps in cholestatic conditions comprise a thorough patient history, abdominal imaging and distinct serological studies including antimitochondrial antibodies and IgG4 levels; if the diagnosis remains unclear, liver biopsy is warranted. Genetic testing should also be considered, as mutations in the hepatobiliary transporters ATP8B1, ABCB11 and ABCB4 are causative for three different forms of familial intrahepatic cholestasis. Disease severity is dependent on the genotypic variants of these transporters, ranging from mildly elevated liver enzymes in adults to cirrhosis in early childhood. Ligands of nuclear receptors, which represent important regulators of hepatobiliary transporters, and modified bile salts are new promising therapeutic options in cholestatic liver disease and are currently being investigated in clinical trials.

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EASL Clinical Practice Guidelines: Management of cholestatic liver diseases

Cited by 1,484 publications

References 261 publications

Dominant strictures in primary sclerosing cholangitis: A multicenter survey of clinical definitions and practices

Dominant strictures in primary sclerosing cholangitis: A multicenter survey of clinical definitions and practices

Genetic Risk Factors for Autoimmune Thyroid Disease might Affect the Susceptibility to and Modulate the Progression of Primary Biliary Cholangitis

Cholestatic Liver Disease

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