AASLD practice guidance on primary sclerosing cholangitis and cholangiocarcinoma

Bowlus, Christopher L.; Arrivé, Lionel; Bergquist, Annika; Deneau, Mark; Forman, Lisa; Ilyas, Sumera; Lunsford, Keri E.; Martínez, Mercedes; Sapisochín, Gonzalo; Shroff, Rachna T.; Tabibian, James H.; Assis, David N.

doi:10.1002/hep.32771

Cited by 94 publications

(130 citation statements)

References 520 publications

(1,226 reference statements)

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“…The types of hepatobiliary malignancies included in this study were cholangiocarcinoma, hepatocellular carcinoma, and gallbladder cancer. This is in line with the current guidelines for surveillance recommendations of hepatobiliary cancers in patients with PSC [ 3 , 15 ].…”

Section: Methodssupporting

confidence: 88%

“…This retrospective study was reviewed and accepted by the Institutional Review Board of the University of Alberta. Patients diagnosed with PSC, according to the European Association for the Study (EASL) guidelines [ 3 ] and the American Association for Study of the Liver (AASLD) practice guidance [ 15 ], were included. Patients assessed and monitored by the Autoimmune Liver Disease Clinic at the University of Alberta, Edmonton, Canada from 1972–2019 were initially included ( n = 455).…”

Section: Methodsmentioning

confidence: 99%

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Prognostic Significance of Severe Vitamin D Deficiency in Patients with Primary Sclerosing Cholangitis

et al. 2023

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Vitamin D deficiency has been linked with adverse events in various liver diseases. The present study aimed to recognize the association between severe vitamin D deficiency and disease progression, hepatobiliary malignancies, liver-related mortality, and the need for liver transplantation in primary sclerosing cholangitis (PSC). Patients with a diagnosis of PSC (n = 354), followed by the autoimmune liver disease clinic at the University of Alberta, were included. Patients with vitamin D levels < 25 nmol/L were defined as severely deficient. Univariate and multivariate analyses were conducted using the Cox proportional hazards regression models. The mean vitamin D level was 59 ± 2 nmol/L, and 63 patients (18%) had a severe vitamin D deficiency. Patients with a severe vitamin D deficiency were 2.5 times more likely to experience hepatobiliary malignancies (HR 2.55, 95% CI, 1.02–6.40, p = 0.046). A severe vitamin D deficiency at diagnosis (HR 1.82, 95% CI, 1.05–3.15, p = 0.03) and persistent deficiencies over time (HR 2.26, 95% CI, 1.17–4.37, p = 0.02) were independently associated with a higher risk of poor clinical liver outcomes. A severe vitamin D deficiency at diagnosis and persistent deficiency at longitudinal assessments were associated with liver-related mortality or the need for liver transplantation.

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Section: Methodssupporting

confidence: 88%

Section: Methodsmentioning

confidence: 99%

Prognostic Significance of Severe Vitamin D Deficiency in Patients with Primary Sclerosing Cholangitis

et al. 2023

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“…Therefore, surveillance for CCA through some form of noninvasive imaging is recommended for this population, especially in the first year of PSC diagnosis ( 12 , 13 ). ERCP is recommended to further evaluate abnormalities in imaging or biomarkers in patients with PSC undergoing surveillance ( 13 , 14 ). Of interest, our study also showed that patients with de novo CCA had a significantly increased presence of mass on imaging and higher CA 19-9 level during biliary brushing when compared with patients with PSC CCA.…”

Section: Discussionmentioning

confidence: 99%

Evaluating the Significance of Pancreatobiliary Fluorescence In Situ Hybridization Polysomy on Prognosis in De Novo Cholangiocarcinoma

Fritcher

Yin

et al. 2022

Clinical and Translational Gastroenterology

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INTRODUCTION: We recently developed a fluorescence in situ hybridization probe set for evaluating suspicious biliary and pancreatic duct strictures (PB-FISH). We aimed to determine whether PB-FISH results in biliary brush cytology specimens are associated with outcomes of patients with cholangiocarcinoma (CCA). METHODS: We performed a retrospective study of patients with CCA tested by PB-FISH from January 2015 to August 2018. CCA was stratified by primary sclerosing cholangitis (PSC) into those with (PSC CCA) or without PSC ( de novo CCA). PB-FISH results were categorized as polysomy (gain of multiple loci), nonpolysomy (single locus gain, single locus gain with 9p21 loss, homozygous 9p21 loss, tetrasomy), and disomy (no abnormalities). Overall survival (OS) was estimated using Kaplan-Meier methods and compared between the PB-FISH results using log-rank tests. Cox models were adjusted for age, sex, CA 19-9, cytology results, source of brushing sample, and treatments. RESULTS: Characteristics of 264 eligible patients (median age 60.4; range 18–92) were comparable for patients with PB-FISH polysomy vs nonpolysomy vs disomy. The median OS was similar between disomy, nonpolysomy, and polysomy in the overall population (22.7 vs 22.7 vs 20.3 months, respectively). For de novo CCA, both polysomy and nonpolysomy were associated with worse OS compared with disomy (polysomy: hazard ratio [HR] = 2.09, 95% confidence interval [CI] = 1.14–3.83; nonpolysomy: HR = 2.4, 95% CI = 0.54–2.46; P = 0.027). For PSC CCA, neither polysomy nor nonpolysomy were significantly associated with worse OS (polysomy: 0.90, 95% CI = 0.47–1.75; nonpolysomy: HR = 1.78, CI = 0.71–4.49; P = 0.27). DISCUSSION: PB-FISH alterations are associated with worse survival in de novo CCA, though statistical significance was lost when adjusting for confounding variables.

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“…Liver transplantation is an option for patients with late-stage cholestatic liver disease, both due to liver disease and for severe, treatmentresistant itch. 5,15,16 Case Study 1: Primary Biliary Cholangitis…”

Section: Meeting Summarymentioning

confidence: 99%

“…27 Itch treatment should include practical recommendations such as avoiding heat, including hot water, tight or itchy clothing, and avoiding emollients. 15,16,28 Current medications include anion exchange resins (e.g., cholestyramine), usually as the first-line choice; opioid receptor antagonists (e.g., naltrexone); pregnane X receptor agonists (e.g., rifampicin); fibrates (e.g., bezafibrate); antihistamines; SSRIs; and, in PFIC, ileal bile acid transporter inhibitors (e.g., odevixibat). However, clinical management guidelines regarding the choice of treatment may differ between types of cholestatic liver disease and what has been approved for each condition.…”

Section: Treatment For Cholestatic Itchmentioning

confidence: 99%

Management of Clinically Significant Itch in Cholestatic Liver Disease

et al. 2023

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Cholestatic liver diseases include primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), and progressive familial intrahepatic cholestasis (PFIC). In all of these conditions, cholestatic itch is a major symptom that can severely and chronically impact a person’s quality of life (QoL). At a satellite symposium presented at the 2022 meeting of the American Association for the Study of Liver Diseases (AASLD) in Washington, D.C., USA, leading experts discussed the importance of assessing itch in all patients with one of these cholestatic liver diseases. The experts presented patient cases to illustrate the challenges of managing itch in these cholestatic liver diseases. Studies show that many of these patients are not being adequately treated for this important symptom. However, while there are several treatments for itch, although not all are specifically approved medications, finding the right one for each patient may be a process of trial and error. In some cases, for people with severe, chronic, non-treatment-responsive cholestatic itch, a liver transplant may be the only treatment option.

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AASLD practice guidance on primary sclerosing cholangitis and cholangiocarcinoma

Cited by 94 publications

References 520 publications

Prognostic Significance of Severe Vitamin D Deficiency in Patients with Primary Sclerosing Cholangitis

Prognostic Significance of Severe Vitamin D Deficiency in Patients with Primary Sclerosing Cholangitis

Evaluating the Significance of Pancreatobiliary Fluorescence In Situ Hybridization Polysomy on Prognosis in De Novo Cholangiocarcinoma

Management of Clinically Significant Itch in Cholestatic Liver Disease

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