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Objective: Primary sclerosing cholangitis is a severe liver disease. Liver transplantation is the only curative therapeutic option. The unpredictable disease course causes much uncertainty and anxiety among patients and relatives. Improved disease knowledge may result in better health outcomes. In PSC, there is lack of high quality patient education materials. The aim of this study was to evaluate the ability of a 3-dimensional education video to improve PSC knowledge in patients and relatives., Methods: A digital survey containing questions about PSC, anxiety and satisfaction was sent prior to, directly after, and one week after watching the video. Both European and American patients and relatives were included., Results: A total of 278 participants (224 patients and 54 relatives) were included. PSC knowledge score increased from 53 % to 74 % directly after and 70 % one week after the video. The STAI anxiety score decreased after the video (-0,8, p = 0,007). Younger age and lower baseline knowledge were independent predictors of knowledge improvement., Conclusion: Disease knowledge improved after watching the video and this was sustained one week later. Generally, patients were very enthusiastic about the video., Practice Implications: 3D education videos can be useful to increase disease knowledge in a severe disease such as PSC., (Copyright © 2020. Published by Elsevier B.V.)

Primary sclerosing cholangitis (PSC) is strongly associated with several human leukocyte antigen (HLA) haplotypes. Due to extensive linkage disequilibrium and multiple polymorphic candidate genes in the HLA complex, identifying the alleles responsible for these associations has proven difficult. We aimed to evaluate whether studying populations of admixed or non-European descent could help in defining the causative HLA alleles. When assessing haplotypes carrying HLA-DRB1*13:01 (hypothesized to specifically increase the susceptibility to chronic cholangitis), we observed that every haplotype in the Scandinavian PSC population carried HLA-DQB1*06:03. In contrast, only 65% of HLA-DRB1*13:01 haplotypes in an admixed/non-European PSC population carried this allele, suggesting that further assessments of the PSC-associated haplotype HLA-DRB1*13:01-DQA1*01:03-DQB1*06:03 in admixed or multi-ethnic populations could aid in identifying the causative allele., (© 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background and Study Aims: We previously developed a prognostic model for primary sclerosing cholangitis (PSC), which was primarily based on a cholangiographic classification of the intra- and extrahepatic biliary tree lesions. The aim of the present study was to validate the performance of this model in an external cohort., Patients and Methods: The validation dataset consisted of patients with PSC from a single referral center in Oslo, Norway. The patients' cholangiograms were scored according to the Amsterdam classification. We then examined whether adjusting the value of the original coefficients of the predictors or adding new predictors would improve the fit of the original model in the validation cohort. In addition, we evaluated calibration (closeness between observed and expected survival) and discrimination using the concordance index., Results: A total of 111 patients (mean age 35 +/- 13 years; 76 % male) were included in the validation study. Baseline clinical characteristics were comparable between the two cohorts. None of the coefficients that were re-estimated in the validation cohort differed significantly from the values of the original model. Observed and expected survival curves were in close agreement across different risk groups. Discrimination of the original model was preserved in the validation cohort: the concordance index was the same in both cohorts., Conclusions: The prognostic model showed adequate performance in an independent series of patients. Therefore, we updated the model using the data from both cohorts to provide more robust estimates of transplant-free survival for individual patients. A nomogram was constructed, which can be used to predict medium- and long-term prognosis in individual patients with PSC., (Copyright Georg Thieme Verlag KG Stuttgart . New York.)

Background: Median survival of patients with primary sclerosing cholangitis (PSC) has been estimated to be 12 years. Cholangiography is the gold standard for diagnosis but is rarely used in estimating prognosis., Aims: To assess the natural history of Dutch PSC patients and to evaluate the prognostic value of a cholangiographic classification system., Patients: A total of 174 patients with established PSC attending a university hospital and three teaching hospitals from 1970 to 1999., Methods: Charts were reviewed for validity and time of diagnosis, concurrent inflammatory bowel disease, interventions, liver transplantation, occurrence of cholangiocarcinoma, and death. Follow up data were obtained from the charts and from the attending clinician or family physician. Median follow up was 76 months (range 1-300). The earliest available cholangiography was scored using a radiological classification system for the severity of sclerosis, developed in our institution. Survival curves were computed by the Kaplan-Meier method. Cholangiographic staging was used to construct a prognostic model, applying Cox proportional hazards analysis., Results: The estimated median survival from time of diagnosis to death from liver disease or liver transplantation was 18 years. Cholangiocarcinoma was found in 18 (10%) patients. Fourteen patients (8%) underwent liver transplantation. Cholangiographic scoring was inversely correlated with survival. A combination of intrahepatic and extrahepatic scoring, together with age at endoscopic retrograde cholangiopancreatography, proved strongly predictive of survival., Conclusions: The observed survival was considerably better than reported in earlier series from Sweden, the UK, and the USA. Classification and staging of cholangiographic abnormalities has prognostic value.

Background: There is no uniformly accepted classification system for the range of cholangiographic abnormalities encountered in primary sclerosing cholangitis (PSC). The aims of this study were to evaluate a previously developed classification system and to test the hypothesis that the pancreatic duct can be involved in PSC., Methods: Two observers scored 132 endoscopic retrograde cholangiopancreatographies (ERCPs) from established PSC patients. From 30 patients, subsequent ERCPs were scored and compared with the initial ERCPs. The pancreatic duct was judged with regard to morphologic abnormalities., Results: The classification system was applicable in 107 patients. In 10 ERCPs (7.6%), no clear intrahepatic abnormalities were found; 15 other ERCPs (11.4%) did not show extrahepatic abnormalities. In 30 cases, a subsequent ERCP was judged. The difference in scoring between the initial and the subsequent ERCPs was statistically significant, with the subsequent ERCP having higher intrahepatic and extrahepatic scores. Sixty-four adequately filled pancreatic ducts were analyzed. In two cases (3.1%), morphologic abnormalities were found., Conclusions: The previously developed scoring system is very applicable for almost all PSC patients when supplemented with a type 0 category. Scoring increased over time, suggesting a correlation with disease severity. The pancreatic duct does not seem to be involved in PSC.

Objective: Symptomatic dominant strictures in primary sclerosing cholangitis are often treated with endoscopic stent therapy, but the optimal treatment duration is not well established. After a promising pilot study, we now report our 4 yr experience with short term endoscopic stent therapy for relief of dominant strictures., Methods: Between January 1994 and October 1997, 32 patients with symptomatic primary sclerosing cholangitis with a dominant stricture at endoscopic retrograde cholangiopancreatography were treated with insertion of a 7- or 10-Fr polyethylene endoprosthesis, which was extracted after a mean of 11 days (range 1-23 days). Primary end points were changes in complaints and cholestasis after 2 months, and time interval until a repeat endoscopic treatment was deemed necessary. A secondary end point was the occurrence of treatment-related complications., Results: Cholestatic complaints improved after 2 months in 83% of patients. Mean scores for pruritus, fatigue, and right upper quadrant pain decreased from 0.94, 1.0, and 0.87 to 0.26, 0.39, and 0.26, respectively. All improvements were significant. Of 14 patients presenting with jaundice, 12 regained normal serum bilirubin levels 2 months after short term endoscopic stenting. The mean levels of conjugated bilirubin, alkaline phosphatase, and gamma-glutamyl transpeptidase dropped significantly from 36 micromol/L, 309 U/L, and 426 U/L to 7 micromol/L, 205 U/L, and 258 U/L, respectively. The reintervention-free proportions after 1 and 3 yr were 80% and 60%. Seven transient procedure-related complications occurred in 45 therapeutic endoscopic retrograde cholangiopancreatographies., Conclusions: Short term endoscopic stenting for symptomatic dominant strictures in primary sclerosing cholangitis is effective and safe, and the beneficial effect is sustained for several years.

Objectives: There are only limited data about the nature of the mononuclear infiltrate surrounding the affected biliary canaliculi in primary sclerosing cholangitis (PSC). The aim of this study was to characterize the composition of the mononuclear infiltrate and to detect signs of activation/ proliferation among the various subpopulations involved. Furthermore the putative role of the biliary epithelium as antigen presenting cells (APC) was assessed., Methods: Liver biopsies of 14 PSC patients were analysed. Seven liver specimens of non-inflammatory liver disease (NIL) patients with hepatocellular carcinoma or metastasis from colorectal carcinoma, as well as eight liver biopsies of primary biliary cirrhosis (PBC) patients, served as controls. Paraffin embedded material was stained with GB7, anti-CD3, anti-CD20, anti-CD45RO. Deep frozen sections were stained with anti-CD4, anti-CD8, anti-CD25, anti-CD86, anti-HLA-DR, anti-IFNgamma, anti-IL4, anti-ICAM1 and anti-alpha4beta7. Stainings were scored by two pathologists using a semiquantitative scale., Results: The portal infiltrate was found to consist mainly of CD3+CD45RO+ cells. Few cells expressed activation or proliferation markers in any of the liver specimens. In the PSC-material, significantly more of the infiltrative lymphocytes were positive for the integrin alpha4beta7, as compared to hardly any positive cells in the NIL-group (P < 0.001) and < 10% in the PBC-specimens (P < 0.01). Variable HLA-DR expression of the biliary epithelium was observed in all groups, however, without co-expression of ICAM1 or B7.2., Conclusions: The portal infiltrate in PSC liver histology specimens appears to consist mainly of non-activated memory T-lymphocytes, a substantial proportion of which expresses the gut-homing integrin alpha4beta7. An antigen-presenting role for the biliary epithelium could not be demonstrated.

Background and Study Aims: Around 10% of patients with primary sclerosing cholangitis (PSC) develop cholangiocarcinoma, which is cholangiographically often indistinguishable from a benign dominant stricture. The aim of the present study was to assess the value of brush cytology in discriminating between benign and malignant dominant strictures in primary sclerosing cholangitis., Patients and Methods: The results of all brush cytology specimens from dominant strictures from patients with established primary sclerosing cholangitis, taken at endoscopic retrograde cholangiopancreatography between 1987 and 1996, were compared with the histological diagnosis or clinical status of the patients at least 2 years later., Results: A total of 47 brush cytology samples, taken between 1987 and 1996, from 43 PSC patients could be included. Between 1993 and 1996, p53 immunocytochemical examination was done in 27 brush cytology specimens and K-ras mutation analysis in 25 patients. The sensitivity, specificity, positive predictive value, and negative predictive value of brush cytology for detection of malignancy were 60%, 89%, 59%, and 89%, respectively. These figures were not improved by adding the results of p53 and K-ras analysis. Logistic regression analysis did not reveal any additional benefit of p53 or K-ras analysis either. Prior stenting did not adversely affect specificity., Conclusions: The sensitivity and positive predictive value of brush cytology for dominant strictures in PSC are rather poor. The specificity and negative predictive value are reasonably good. There was no additional value from p53 immunocytochemistry and K-ras mutation analysis. Prior stenting did not affect the results.