ESGE/EASL recommend that, as the primary diagnostic modality for PSC, magnetic resonance cholangiography (MRC) should be preferred over endoscopic retrograde cholangiopancreatography (ERCP).Moderate ...quality evidence, strong recommendation.
ESGE/EASL suggest that ERCP can be considered if MRC plus liver biopsy is equivocal or contraindicated in patients with persisting clinical suspicion of PSC. The risks of ERCP have to be weighed against the potential benefit with regard to surveillance and treatment recommendations.Low quality evidence, weak recommendation.
ESGE/EASL suggest that, in patients with an established diagnosis of PSC, MRC should be considered before therapeutic ERCP.Weak recommendation, low quality evidence.
ESGE/EASL suggest performing endoscopic treatment with concomitant ductal sampling (brush cytology, endobiliary biopsies) of suspected significant strictures identified at MRC in PSC patients who present with symptoms likely to improve following endoscopic treatment.Strong recommendation, low quality evidence.
ESGE/EASL recommend weighing the anticipated benefits of biliary papillotomy/sphincterotomy against its risks on a case-by-case basis.Strong recommendation, moderate quality evidence.Biliary papillotomy/sphincterotomy should be considered especially after difficult cannulation.Strong recommendation, low quality evidence.
ESGE/EASL suggest routine administration of prophylactic antibiotics before ERCP in patients with PSC.Strong recommendation, low quality evidence.
EASL/ESGE recommend that cholangiocarcinoma (CCA) should be suspected in any patient with worsening cholestasis, weight loss, raised serum CA19-9, and/or new or progressive dominant stricture, particularly with an associated enhancing mass lesion.Strong recommendation, moderate quality evidence.
ESGE/EASL recommend ductal sampling (brush cytology, endobiliary biopsies) as part of the initial investigation for the diagnosis and staging of suspected CCA in patients with PSC.Strong recommendation, high quality evidence.
Background & Aims The biochemical response to ursodeoxycholic acid (UDCA) in primary biliary cirrhosis (PBC) correlates with the long-term prognosis and thus could allow the identification of the ...patients needing new therapeutic approaches. Due to variation in both endpoints and studied populations, there is still no full agreement on the definition of the biochemical response. The aim of our study was to determine, in a population of patients with only early-stage disease, the best biochemical criteria of response to UDCA allowing to predict the absence of poor outcome, as defined by liver-related death, liver transplantation, complications of cirrhosis, or histological evidence of cirrhosis development. Methods The efficiency of several combinations of serum bilirubin, alkaline phosphatase (ALP), and aspartate aminotransferase (AST) threshold values to predict outcome was assessed after 1 year of UDCA in 165 patients with early-stage PBC followed up for an average 7 years. The Barcelona, Paris, Rotterdam, and Toronto criteria were also assessed. Results The most accurate discrimination of the patients according to the multiple endpoints was given by the following criteria: ALP and AST ⩽1.5× upper limit of normal, with a normal bilirubin level. Responders and non-responders were equally distributed, while all adverse events were observed in non-responders ( p <0.001). These criteria remained valid when early PBC was defined by both normal bilirubin and albumin concentrations at baseline. Conclusions This study defines the best efficient biochemical response to UDCA that identifies patients with early PBC at very low risk of long-term development of liver failure or cirrhosis.
Chazouilleres expresses compliment on Fickert et al on their study of double blind randomized, placebo-controlled study evaluating the safety and efficacy of 24-norursodeoxycholic acid (norUDCA) in ...patients with primary sclerosing cholangitis (PSC). The study demonstrated the role of norUDCAin reducing alkaline phosphatase (ALP) values in a dose-dependent manner. The effect is associated with significant decrease in other liver enzyme levels but not in serum bilirubin values. He added the safety profile of norUDCA was unremarkable, the pruritus did not change and there was no effect on inflammatory bowel disease (IBD). He explores the aspects of possible application of norUDCA in the future treatment of PSC.
Patients with primary sclerosing cholangitis (PSC) develop progressive liver fibrosis and end-stage liver disease. Non-invasive and widely available parameters are urgently needed to assess disease ...stage and the risk of clinical progression. Transient elastography (TE) has been reported to predict fibrosis stage and disease progression. However, these results have not been confirmed in an independent cohort and comparison of TE measurement to other non-invasive means is missing.
In a retrospective study we collected data from consecutive PSC patients receiving TE measurements from 2006 to 2014 (n = 139). Data from 62 patients who also underwent a liver biopsy were used to assess the performance of TE and spleen length (SL) measurement for the staging of liver fibrosis. Follow-up data from this cohort (n = 130, Hamburg) and another independent cohort (n = 80, Paris) was used to compare TE and SL as predictors of clinical outcome applying Harrel's C calculations.
TE measurement had a very good performance for the diagnosis and exclusion of higher fibrosis stages (≥F3: AUROC 0.95) and an excellent performance for the diagnosis and exclusion of cirrhosis (F4 vs. < F4: AUROC 0.98). Single-point TE measurement had very similar predictive power for patient outcome as previously published. In a combined cohort of PSC patients (n = 210), SL measurements had a similar performance as TE for the prediction of patient outcome (5 x cross-validated Harrel's C 0.76 and 0.72 for SL and TE, respectively).
Baseline TE measurement has an excellent performance to diagnose higher fibrosis stages in PSC. Baseline measurements of SL and TE have similar usefulness as predictive markers for disease progression in patients with PSC.
In patients with mild hepatitis C, the usefulness of antiviral therapy is subject of debate, as a low risk for progression of fibrosis is assumed. Several studies have shown that steatosis is a ...strong and independent predictor of the severity as well as the progression of fibrosis in chronic hepatitis C. Therefore, this study assessed the impact of steatosis on the progression of fibrosis between paired liver biopsies in untreated patients with mild hepatitis on index biopsy. One hundred thirty-five untreated patients (mean age, 38 years; M/F sex ratio, 1.43) with one known risk factor of infection (68 transfusions, 67 injecting drug use) had 2 liver biopsies after a median interval of 61 months (18-158). All had METAVIR score of A1F1 or lower at first liver biopsy. Unequivocal progression of fibrosis was considered if patients had a fibrosis score of 3 or 4 at the second liver biopsy. The probability of progression of fibrosis was estimated by using the Kaplan-Meier method. During follow-up, progression of fibrosis occurred in 21 patients (16%) after a median delay of 65 months. Cumulative probabilities of the progression of fibrosis at 4 and 6 years were 5.2% and 19.8%, respectively. In multivariate analysis, steatosis was the only independent factor predictive of progression of fibrosis (RR, 4.8; CI, 1.3-18.3). Probability of progression of fibrosis was significantly related to the percentage of hepatocytes with steatosis. In conclusion, steatosis is a major determinant of the progression of fibrosis in mild hepatitis C, regardless of the genotype. Our results argue for antiviral treatment in the subgroup of patients with mild hepatitis and steatosis.
AIM： To investigate the phenotype of inflammatory bowel disease associated with primary sclerosing cholangitis （PSC-IBD）.
METHODS： Data from 75 PSC-IBD patients evaluated in our tertiary center ...between 1963 and 2006 were collected and compared to 150 IBD patients without PSC, matched for sex, birth date, IBD diagnosis date and initial disease location regarding ileal, different colonic segments, and rectum, respectively.
RESULTS： While PSC-IBD patients received more 5-aminosalicylates （8.7 years/patient vs 2.9 years/ patient, P 〈 0.001）, they required less immuno-suppressors （24% vs 46% at 10 years; P 〈 0.001） and less intestinal resection （10% vs 44% at 10 years, P 〈 0.001）. The 25-year cumulative rate of colectomy was 25.1% in PSC-IBD and 37.3% in controls （P = 0.004）. The 25-year cumulative rate of colorectal cancer was 23.4% in PSC-IBD vs 0% in controls （P = 0.002）. PSC was the only independent risk factor for the development of colorectal cancer （OR = 10.8; 95% CI, 3.7-31.3）. Overall survival rate without liver transplantation was reduced in PSC-IBD patients （67% vs 91% in controls at 25 years, P = 0.001）.
CONCLUSION： This study confirms that patients with PSC-IBD have a particular disease phenotype independent of the initial disease location. Although their disease is less active and they use more 5-aminosalicylates, they present a higher risk of colorectal cancer.
Data on the management of primary sclerosing cholangitis (PSC) in European expert centres are sparse. In this study, a PSC group from the ERN RARE-LIVER surveyed European hepatologists to uncover ...differences in real-life clinical practices.
In April 2020 a survey questionnaire was sent to members of the International PSC Study Group and ERN RARE-LIVER. Participants were asked about the size of their PSC cohort, use of medical treatments including ursodeoxycholic acid (UDCA) and surveillance for cholangiocarcinoma, gallbladder polyps and inflammatory bowel disease (IBD). Data were presented descriptively.
Eighty-two of 278 members responded. Fifty percent of physicians prescribed UDCA routinely to all their patients with PSC, whereas 12% never prescribed UDCA. UDCA was used for one or more indications including: alkaline phosphatase >1.5x the upper limit of normal, severe PSC changes, pruritus, PSC-IBD or patient demand. Few physicians offered other medical treatments than UDCA. The use of medical treatments was generally comparable in small (<99 patients) and large (≥99 patients) cohorts, as well as for adult and paediatric physicians. Most physicians routinely screened for cholangiocarcinoma and the most frequent modalities used were MRI and ultrasound. At detection of a gallbladder polyp of 6 mm, 46% of physicians recommended repeated ultrasound after 3-6 months, whereas 44% of physicians recommended immediate cholecystectomy. In patients with PSC without IBD at PSC diagnosis, 68% of physicians repeated colonoscopy within 3-5 years whereas 27% referred only patients who developed symptoms of IBD.
Substantial variations in treatment and monitoring of European patients with PSC were discovered. Harmonisation of strategies is desirable to enable improved interpretation of outcome data and to optimise clinical patient care.
In this study, we explored how different centres in Europe manage primary sclerosing cholangitis (PSC), a rare inflammatory disease of the bile ducts. We collected information through a questionnaire sent to specialist physicians who were part of a European network for rare liver diseases. We found several differences in how patients with PSC were monitored and treated. This includes differences in surveillance for bile duct cancer, gallbladder polyps and inflammatory bowel disease. By pointing out these differences, we hope that management of PSC will be standardized, which could aid clinical research and benefit patients.
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•Substantial variations in the treatment and monitoring of patients with PSC was seen across Europe.•Considerable discrepancies between practice and published guidelines in the management of patients with PSC existed.•Despite no robust evidence or clear recommendations, most physicians treated all their patients with UDCA.•Regular screening for cholangiocarcinoma was performed by 90% of physicians. A variety of screening methods were used.•In PSC without IBD detected at diagnosis of the bile duct disease, most physicians would repeat colonoscopy on a regular basis.
•We explored the pathways leading to hepatocellular carcinoma in hepatitis B disease.•The relationships between clinical characteristics and the cancer were analyzed.•Liver fibrosis has a key role in ...the occurrence of hepatocellular carcinoma.•The risk of occurrence is increased among elderly men with a metabolic syndrome.•Excessive alcohol consumption is also associated with a higher risk.
The factors predicting hepatocellular carcinoma (HCC) occurrence in chronic hepatitis B need to be precisely known to improve its detection. We identified pathways and individual predictive factors associated with HCC in the ANRS CO22 HEPATHER cohort.
The study analyzed HBV-infected patients recruited at 32 French expert hepatology centers from August 6, 2012, to December 31, 2015. We excluded patients with chronic HCV, HDV and a history of HCC, decompensated cirrhosis or liver transplantation. Structural equation models were developed to characterize the causal pathways leading to HCC occurrence. The association between clinical characteristics (age, gender, body-mass index, liver fibrosis, alcohol consumption, smoking status, diabetes, hypertension, dyslipidemia, alpha-fetoprotein, HBV DNA levels, antiviral therapy) and incident HCC was quantified.
Among the 4489 patients included, 33 patients reported incident HCC. The median follow-up was 45.5 months. Age (β = 0.18 by decade, 95% CI 0.14−0.23), male gender (β = 0.23, 95% CI 0.18−0.29), metabolic syndrome (β = 0.28, 95% CI 0.22−0.33), alcohol consumption (β = 0.09, 95% CI 0.05−0.14) and HBV DNA (β = 0.25, 95% CI 0.170.34) had a significant and direct effect on the occurrence of advanced liver fibrosis. Liver fibrosis (β = 0.71, 95% CI 0.55−0.87) predicted, in turn, the occurrence of HCC.
Liver fibrosis mediates the effects of age, gender, alcohol, metabolic syndrome and HBV DNA on the occurrence of HCC. Elderly men with chronic hepatitis B, risky alcohol use, advanced liver fibrosis, metabolic syndrome and high HBV DNA levels should be monitored closely to detect the development of HCC.
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