scholarly journals Systematic Review of Prognostic Models Compared to the Mayo Risk Score for Primary Sclerosing Cholangitis

2021 ◽  
Vol 10 (19) ◽  
pp. 4476
Author(s):  
Paul A. Schmeltzer ◽  
Mark W. Russo
Keyword(s):  
Systematic Review ◽  
Liver Disease ◽  
Clinical Outcomes ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Prognostic Models ◽  
Free Survival ◽  
Clinical Endpoints ◽  
Non Invasive ◽  
The Uk

Background: Primary sclerosing cholangitis (PSC) is a cholestatic liver disease with a variable clinical course that can ultimately lead to end-stage liver disease, cholangiocarcinoma, and the need for liver transplantation. Several prognostic models have been developed to predict clinical outcomes and have been compared to the revised Mayo Risk Score (rMRS). Aim: To conduct a systematic review comparing the rMRS to other non-invasive prognostic tests for PSC. Methods: A systematic review of studies from 2000 to 2020 was performed that compared non-invasive biochemical prognostic models to the rMRS in predicting outcomes in patients with PSC. Results: Thirty-seven studies were identified, of which five studies that collectively included 3230 patients were reviewed. Outcomes included transplant-free survival or composite clinical outcomes. The rMRS was better than the Amsterdam–Oxford model for predicting 1-year transplant-free survival, c-statistics 0.75 and 0.70, respectively. The UK-PSC score outperformed the rMRS for 10-year transplant-free survival, c-statistics 0.85 and 0.69, respectively. An enhanced liver fibrosis score was independently associated with transplant-free survival after adjusting for rMRS. PREsTo predicts 5-year hepatic decompensation with a c-statistic modestly higher than rMRS; 0.90 and 0.85, respectively. Conclusion: Newer prognostic models, including the UK-PSC score and PREsTo, are more accurate at predicting clinical endpoints in PSC compared to the rMRS. Time frames and clinical endpoints are not standard among studies.

scholarly journals Quality of life in primary sclerosing cholangitis: a systematic review

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Elena Marcus ◽  
Paddy Stone ◽  
Anna-Maria Krooupa ◽  
Douglas Thorburn ◽  
Bella Vivat
Keyword(s):  
Quality Of Life ◽  
Systematic Review ◽  
Clinical Research ◽  
Disease Progression ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Small Sample ◽  
Limited Evidence ◽  
Research Studies

Abstract Background Primary sclerosing cholangitis (PSC) is a rare bile duct and liver disease which can considerably impact quality of life (QoL). As part of a project developing a measure of QoL for people with PSC, we conducted a systematic review with four review questions. The first of these questions overlaps with a recently published systematic review, so this paper reports on the last three of our initial four questions: (A) How does QoL in PSC compare with other groups?, (B) Which attributes/factors are associated with impaired QoL in PSC?, (C) Which interventions are effective in improving QoL in people with PSC?. Methods We systematically searched five databases from inception to 1 November 2020 and assessed the methodological quality of included studies using standard checklists. Results We identified 28 studies: 17 for (A), ten for (B), and nine for (C). Limited evidence was found for all review questions, with few studies included in each comparison, and small sample sizes. The limited evidence available indicated poorer QoL for people with PSC compared with healthy controls, but findings were mixed for comparisons with the general population. QoL outcomes in PSC were comparable to other chronic conditions. Itch, pain, jaundice, severity of inflammatory bowel disease, liver cirrhosis, and large-duct PSC were all associated with impaired QoL. No associations were found between QoL and PSC severity measured with surrogate markers of disease progression or one of three prognostic scoring systems. No interventions were found to improve QoL outcomes. Conclusion The limited findings from included studies suggest that markers of disease progression used in clinical trials may not reflect the experiences of people with PSC. This highlights the importance for clinical research studies to assess QoL alongside clinical and laboratory-based outcomes. A valid and responsive PSC-specific measure of QoL, to adequately capture all issues of importance to people with PSC, would therefore be helpful for clinical research studies.

Severity of liver disease does not predict osteopenia or low bone mineral density in primary sclerosing cholangitis

2005 ◽  
Vol 25 (2) ◽  
pp. 311-316 ◽  
Author(s):  
Mical S. Campbell ◽  
Gary R. Lichtenstein ◽  
Andrew D. Rhim ◽  
Michael Pazianas ◽  
Thomas Faust
Keyword(s):  
Bone Mineral Density ◽  
Liver Disease ◽  
Primary Sclerosing Cholangitis ◽  
Bone Mineral ◽  
Sclerosing Cholangitis ◽  
Mineral Density ◽  
Low Bone Mineral Density

scholarly journals Circulating Macrophage Activation Markers Predict Transplant-Free Survival in Patients With Primary Sclerosing Cholangitis

2021 ◽  
Vol 12 (3) ◽  
pp. e00315
Author(s):  
Lars Bossen ◽  
Mette Vesterhus ◽  
Johannes R. Hov ◽  
Martti Färkkilä ◽  
William M. Rosenberg ◽  
...  
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Macrophage Activation ◽  
Activation Markers ◽  
Free Survival

scholarly journals A21 VITAMIN D DEFICIENCY AND ITS ASSOCIATION WITH CLINICAL OUTCOMES IN PRIMARY SCLEROSING CHOLANGITIS

2020 ◽  
Vol 3 (Supplement_1) ◽  
pp. 24-26
Author(s):  
S Wang ◽  
A J Montano-Loza ◽  
M Ebadi
Keyword(s):  
Vitamin D ◽  
Liver Transplantation ◽  
Liver Disease ◽  
Vitamin D Deficiency ◽  
Sclerosing Cholangitis ◽  
Adverse Outcomes ◽  
Vitamin D Status ◽  
Event Free Survival ◽  
Free Survival ◽  
University Of Alberta

Abstract Background Primary sclerosing cholangitis (PSC) is a progressive cholestatic disease involving chronic inflammation and fibrosis of intra- and extra-hepatic ducts. Vitamin D is a secosteroid implicated in anti-inflammatory and anti-fibrotic pathways, and its deficiency has been associated with worse outcomes in chronic liver disease. Vitamin D status may also influence the course of PSC but studies evaluating this link are scarce. Aims To determine the association of vitamin D deficiency with the development of cirrhosis, mortality, and need for liver transplantation in patients with PSC. Methods Ninety-four patients with the diagnosis of PSC were evaluated and followed by the autoimmune liver disease clinic at the University of Alberta, Edmonton, Canada. Clinical data were recovered from medical charts. Vitamin D status was defined by the serum concentration of 25-hydroxyvitamin D3. Patients with levels <50 nmol/L (10 ng/ml) were defined as deficient. Univariate and multivariate analyses were constructed using the Cox proportional hazards regression models. Event-free survival was defined as time from vitamin D assessment to the time of liver transplant or death. Results Mean age at PSC diagnosis was 32±14 years, with 67% of patients being male. The mean vitamin D level was 69±33 nmol/L (range, 4–163 nmol/L) and 26 patients (28%) had vitamin D deficiency (<50 nmol/L). Among 85 patients without cirrhosis at diagnosis, 43 patients (51%) developed cirrhosis. By univariate Cox analysis, serum ALP, albumin, bilirubin and vitamin D deficiency were predictors of cirrhosis development. Vitamin D deficiency was independently associated with higher risk of developing cirrhosis (HR 2.11, 95% CI 1.002–4.44, P=0.049) after adjusting for other predictors. Median time to develop cirrhosis was shorter in patients with vitamin D deficiency (6.8 years; 95% CI, 1.7–11.8) compared to those without (10.8 years; 95% CI, 9.2 -12.4; P=0.007). Over a median follow-up period of 5.6 years, adverse outcomes (liver transplant or death) were observed in 34 patients (36%). Serum levels of albumin, ALP, bilirubin, INR, platelet count, ascites, variceal bleeding and vitamin D deficiency were associated with adverse outcomes in univariate analysis. Vitamin D deficiency was independently associated with higher risk of adverse endpoints (HR 2.87, 95% CI, 1.16–7.12, P=0.02) after adjusting for confounding factors. Event-free survival was shorter in the patients with vitamin D deficiency compared to those without deficiency (7.1 years; 95% CI, 2.4–11.9 vs. 11.4 years; 95% CI, 8.9–13.9, P=0.03, Figure 1). Conclusions Vitamin D deficiency was frequent in patients with PSC and was associated with higher risk of progression to cirrhosis, as well as decreased time to death and liver transplantation. The possibility of improving outcomes in PSC by vitamin D supplementation awaits further investigation. Funding Agencies Food and Health Innovation Initiative (Vitamin Fund), University of Alberta

scholarly journals Systematic review of the impact of non-alcoholic fatty liver disease on mortality and adverse clinical outcomes for individuals with chronic kidney disease

BMJ Open ◽  
2020 ◽  
Vol 10 (9) ◽  
pp. e040970 ◽  
Author(s):  
Theresa Hydes ◽  
Ryan Buchanan ◽  
Oliver J Kennedy ◽  
Simon Fraser ◽  
Julie Parkes ◽  
...  
Keyword(s):  
Systematic Review ◽  
Chronic Kidney Disease ◽  
Liver Disease ◽  
Kidney Disease ◽  
Fatty Liver ◽  
Clinical Outcomes ◽  
Fatty Liver Disease ◽  
Alcoholic Fatty Liver ◽  
All Cause Mortality ◽  
Alcoholic Fatty Liver Disease

ObjectivesTo investigate if non-alcoholic fatty liver disease (NAFLD) impacts mortality and adverse outcomes for individuals with chronic kidney disease (CKD).DesignSystematic review.Data sourcesPubMed, EMBASE and Web of Science were searched up to 1 February 2020 with no restriction on the earliest date.Eligibility criteria for selecting studiesObservational cohort studies that reported either the risk of all-cause mortality, incidence of non-fatal cardiovascular events (CVE) or progression of kidney disease among adults with established CKD who have NAFLD compared with those without.Data extraction and synthesisTwo reviewers extracted data and assessed bias independently.ResultsOf 2604 records identified, 3 studies were included (UK (n=852), South Korea (n=1525) and USA (n=1413)). All were judged to have a low or moderate risk of bias. Data were insufficient for meta-analysis. Two studies examined the influence of NAFLD on all-cause mortality. One reported a significant positive association for NAFLD with all-cause mortality for individuals with CKD (p<0.05) (cardiovascular-related mortality p=ns), which was lost following adjustment for metabolic risk factors; the second reported no effect in adjusted and unadjusted models. The latter was the only study to report outcomes for non-fatal CVEs and observed NAFLD to be an independent risk factor for this (propensity-matched HR=2.00, p=0.02). Two studies examined CKD progression; in one adjusted rate of percentage decline in estimated glomerular filtration rate per year was found to be increased in those with NAFLD (p=0.002), whereas the other found no significant difference.ConclusionsFew studies have examined the influence of NAFLD on prognosis and major adverse clinical outcomes within the CKD population. The studies identified were diverse in design and results were conflicting. This should be a focus for future research as both conditions continue to rise in prevalence and have end-stage events associated with significant health and economic costs.PROSPERO registration numberCRD42020166508.

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