Population-based epidemiology, malignancy risk, and outcome of primary sclerosing cholangitis.

Published on Dec 1, 2013in Hepatology14.971
· DOI :10.1002/HEP.26565
Kirsten Boonstra11
Estimated H-index: 11
Rinse K. Weersma52
Estimated H-index: 52
+ 15 AuthorsCyriel Y. Ponsioen48
Estimated H-index: 48
Extensive population-based studies are much needed to accurately establish epidemiology and disease course in patients with primary sclerosing cholangitis (PSC). We aimed to obtain population-based prevalence and incidence figures, insight in disease course with regard to survival, liver transplantation (LT), and occurrence of malignancies, as well as risk factors thereof. Four independent hospital databases were searched in 44 hospitals in a large geographically defined area of the Netherlands, comprising 50% of the population. In addition, all PSC patients in the three Dutch liver transplant centers and all inflammatory bowel disease (IBD) patients in the adherence area of a large district hospital were identified. All medical records were reviewed on-site, verifying diagnosis. Five hundred and ninety PSC patients were identified, resulting in an incidence of 0.5 and a point prevalence of 6.0 per 100,000. Median follow up was 92 months. Estimated median survival from diagnosis until LT or PSC-related death in the entire cohort was 21.3 years, as opposed to 13.2 years in the combined transplant centers cohort (n = 422; P < 0.0001). Colorectal carcinoma (CRC) risk was 10-fold increased, as compared to ulcerative colitis controls, and developed at a much younger age (39 years; range, 26-64), compared to IBD controls (59 years; range, 34-73; P = 0.019). Colonoscopic surveillance was associated with significantly better outcome. Conclusion: This study exemplifies that, for relatively rare diseases, it is paramount to collect observational data from large, population-based cohorts, because incidence and prevalence rates of PSC are markedly lower and survival much longer than previously reported. The selection of a bias-free, population-based cohort showed a significantly longer survival, compared to the tertiary referral cohort. CRC can develop at an early age, warranting surveillance from time of PSC diagnosis. (Hepatology 2013; 58:2045-2055).
  • References (41)
  • Citations (268)
📖 Papers frequently viewed together
10 Authors (Ulrika Broomé, ..., Greger Lindberg)
606 Citations
471 Citations
684 Citations
78% of Scinapse members use related papers. After signing in, all features are FREE.
#1Vikrant V. Sahasrabuddhe (NIH: National Institutes of Health)H-Index: 28
#2Munira Z. Gunja (UU: Utrecht University)H-Index: 2
Last. Katherine A. McGlynnH-Index: 34
view all 9 authors...
Result s Aspirin users had statistically significant reduced risks of incidence of HCC (RR = 0.59; 95% CI = 0.45 to 0.77) and mortality due to CLD (RR = 0.55; 95% CI = 0.45 to 0.67) compared to those who did not use aspirin. In contrast, users of nonaspirin NSAIDs had a reduced risk of mortality due to CLD (RR = 0.74; 95% CI= 0.61 to 0.90) but did not have lower risk of incidence of HCC (RR = 1.08; 95% CI = 0.84 to 1.39) compared to those who did not use nonaspirin NSAIDs. The risk estimates did...
113 CitationsSource
#1Marcel Janse (UMCG: University Medical Center Groningen)H-Index: 7
#2Laetitia E. Lamberts (UMCG: University Medical Center Groningen)H-Index: 12
Last. Rinse K. Weersma (UMCG: University Medical Center Groningen)H-Index: 52
view all 4 authors...
7 CitationsSource
#1Lisa J. Herrinton (KP: Kaiser Permanente)H-Index: 46
#1Lisa J. Herrinton (KP: Kaiser Permanente)H-Index: 19
Last. Fernando Velayos (UCSF: University of California, San Francisco)H-Index: 28
view all 6 authors...
Background & Aims The relationship between inflammatory bowel disease (IBD) and the incidence and mortality of colorectal adenocarcinoma (CRC) has not been evaluated recently. Methods We calculated the incidence and standardized incidence and mortality rate ratios of CRC among adult individuals with intact colons using Kaiser Permanente of Northern California's database of members with IBD and general membership data for the period of 1998 to June 2010 (data through 2008 were used to calculate m...
167 CitationsSource
#1Tine Jess (SSI: Statens Serum Institut)H-Index: 36
#2Jacob Simonsen (SSI: Statens Serum Institut)H-Index: 27
Last. Morten Frisch (SSI: Statens Serum Institut)H-Index: 60
view all 6 authors...
Background & Aims The risk for colorectal cancer (CRC) in patients with inflammatory bowel disease (IBD) could have changed over time, with changes in treatment options. We studied CRC risk in a nationwide cohort of 47,374 Danish patients with IBD over a 30-year period. Methods We determined relative risk (RR) values using Poisson regression-derived incidence rate ratios of CRC from 1 year after IBD diagnosis, adjusted for age, sex, and calendar time. We compared incidence of CRC among patients ...
296 CitationsSource
#1Kirsten BoonstraH-Index: 11
#2Ulrich BeuersH-Index: 59
Last. Cyriel Y. PonsioenH-Index: 48
view all 3 authors...
Summary Objective Studies on the epidemiology of primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC) show variable outcome. We aimed at systematically reviewing the incidence and prevalence rates, as well as geographical distribution and temporal trends of PSC and PBC. Data sources A systematic search of literature was performed in Medline and EMBASE (search last conducted January 10th, 2011). Study selection Population-based epidemiological studies reporting incidence and/o...
297 CitationsSource
#1Airi JussilaH-Index: 9
#2Lauri J. Virta (Social Insurance Institution)H-Index: 24
Last. Martti Färkkilä (HYKS: Helsinki University Central Hospital)H-Index: 52
view all 6 authors...
Background: The incidence of inflammatory bowel disease (IBD) is high in Western countries, but during the last decade the figures have stabilized, or only slightly increased; at the same time, an increasing incidence rate has been observed in Eastern Europe and Asia. The purpose of this study was to estimate the incidence of IBD in Finland between 2000 and 2007. Methods: New IBD cases between 2000–2007 were retrieved from the national database of special reimbursements for drugs costs. The regi...
42 CitationsSource
#1Enrique Chávez (CINVESTAV)H-Index: 7
#2Luis Castro-Sánchez (CINVESTAV)H-Index: 11
Last. Pablo Muriel (CINVESTAV)H-Index: 34
view all 7 authors...
Non-steroidal anti-inflammatory drugs (NSAIDs) are drugs used primarily to treat inflammation, pain and fever. Their main mechanism of action is cyclooxygenase (COX) inhibition, and this enzyme has been linked to hepatotoxicity. The association of COX and liver injury has been, in part, due to the presence of COX-2 isoform in damaged liver and the possible induction of this enzyme by profibrotic molecules like Transforming Growth Factor-β (TGF-β). The aim of this work was to evaluate the effects...
16 CitationsSource
#1JH Ngu (Canterbury of New Zealand)H-Index: 2
#2Richard B. Gearry (Canterbury of New Zealand)H-Index: 51
Last. Catherine Am Stedman (Canterbury of New Zealand)H-Index: 32
view all 4 authors...
Background & Aims Little is known about the exact etiology of primary sclerosing cholangitis (PSC); epidemiologic data are scarce. We performed a population-based epidemiologic study of PSC in Canterbury, New Zealand. Methods By using multiple case-finding methods, we searched public and private adult and pediatric outpatient clinics, hospital discharge summaries, and radiology and pathology reports to identify all cases of PSC in the region. Cases were included if PSC was identified by endoscop...
64 CitationsSource
#1Laetitia E. Lamberts (UMCG: University Medical Center Groningen)H-Index: 12
#2Marcel Janse (UMCG: University Medical Center Groningen)H-Index: 7
Last. Rinse K. WeersmaH-Index: 52
view all 5 authors...
Abstract Background Primary sclerosing cholangitis is a chronic cholestatic liver disease. An immune aetiology is suggested by associations between PSC and inflammatory bowel disease. Data on concomitant prevalence of other immune-mediated diseases is limited. Aim To assess the prevalence of concomitant immune-mediated diseases and the impact on disease outcome in PSC. Methods We included 241 patients and retrospectively reviewed medical charts. Results Altogether 172 (71.4%) patients had concom...
24 CitationsSource
#1Joana Torres (ISMMS: Icahn School of Medicine at Mount Sinai)H-Index: 19
#2G Pineton de Chambrun (UCSD: University of California, San Diego)H-Index: 7
Last. Jean-Frederic Colombel (ISMMS: Icahn School of Medicine at Mount Sinai)H-Index: 129
view all 5 authors...
Aliment Pharmacol Ther 2011; 34: 497–508 Summary Background Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease strongly associated with inflammatory bowel disease (IBD). IBD patients diagnosed with PSC have an increased risk of colorectal dysplasia and cancer. Aims To review the available evidence regarding colorectal neoplasia epidemiology, preventive strategies and outcomes in patients with PSC and IBD, and to advance some hypotheses regarding possible mechanisms invol...
78 CitationsSource
Cited By268
#1Adam P. Buckholz (Cornell University)
#2Robert S. Brown (Cornell University)H-Index: 78
Cholangiocarcinoma is a highly lethal biliary epithelial tumor that is rare in the general population but has increased rates in patients with primary sclerosing cholangitis (PSC). It is heterogenous, and management varies by location. No effective prevention exists, and screening is likely only feasible in PSC. Patients often present in an advanced state with jaundice, weight loss, and cholestatic liver enzymes. Diagnosis requires imaging with magnetic resonance cholangiopancreatography, labora...
#1C.D. ManganisH-Index: 3
#2Roger W. ChapmanH-Index: 62
Last. Emma L. CulverH-Index: 16
view all 3 authors...
#1Mohammed S. Al Suraih (Mayo Clinic)H-Index: 1
#2Christy E. Trussoni (Mayo Clinic)H-Index: 13
Last. Steven P. O'Hara (Mayo Clinic)H-Index: 24
view all 5 authors...
Background & aims Primary sclerosing cholangitis (PSC) is a chronic liver disease characterized by peribiliary inflammation and fibrosis. Cholangiocyte senescence is a prominent feature of PSC. Here we hypothesize that extracellular vesicles (EVs) from senescent cholangiocytes influence the phenotype of target cells. Methods EVs were isolated from normal human cholangiocytes (NHCs), cholangiocytes from PSC patients, and NHCs experimentally induced to senescence. NHCs, malignant human cholangiocy...
#1Jean-Marc DumonceauH-Index: 53
#2Myriam DelhayeH-Index: 36
Last. Annarita Farina (Geneva College)H-Index: 14
view all 4 authors...
It is frequently challenging to make the correct diagnosis in patients with biliary strictures. This is particularly important as errors may have disastrous consequences. Benign-appearing stricture...
2 CitationsSource
#1Vittorio BranchiH-Index: 7
Last. Steffen ManekellerH-Index: 13
view all 11 authors...
#1Anders Batman Mjelle (University of Bergen)H-Index: 6
#2Guri Fossdal (University of Bergen)
Last. Mette VesterhusH-Index: 16
view all 4 authors...
Abstract The aim of the study described here was to characterize three different liver elastography methods in primary sclerosing cholangitis (PSC) patients, for the first time exploring 2-D shear wave elastography (2-D-SWE) in PSC patients and its putative advantages over point shear wave elastography (pSWE). Sixty-six adult PSC patients (51 males, 77%) underwent liver elastography: Transient elastography (TE), pSWE and 2-D-SWE were applied head-to-head after B-mode ultrasonography and blood te...
#1Vittorio BranchiH-Index: 7
Last. Steffen ManekellerH-Index: 13
view all 11 authors...
Wenngleich in Bezug auf Therapie und Krankheitsverstandnis bei der primar sklerosierenden Cholangitis (PSC) in den vergangenen Jahren erhebliche Fortschritte zu beobachten sind, so ist bei Karzinom und „end-stage liver disease“ (ELD) eine Lebertransplantation (LTX) meist die einzige Chance auf Heilung. In seltenen Fallen ist eine Leberteilresektion (LR) zur kurativen Therapie des PSC-assoziierten Gallengangskarzinoms (CCC) moglich. Diese Operationen stellen fur PSC-Patienten eine zusatzliche Bel...
#1Maaike Biewenga (LUMC: Leiden University Medical Center)H-Index: 1
#2Arantza F. Sarasqueta (UvA: University of Amsterdam)
Last. Leendert A. Trouw (LUMC: Leiden University Medical Center)H-Index: 45
view all 6 authors...
Abstract Introduction The complement system, an essential part of the innate immune system, is involved in various autoimmune diseases. Activation of the complement system by autoantibodies results in immune activation and tissue damage. At the moment little is known about the role of the complement system in autoimmune liver disease, including primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH). Since inhibition of the complement system is curr...
#2Maryam EbadiH-Index: 16
Last. Gurpal SandhaH-Index: 10
view all 3 authors...
#1Magdalena Panek-Jeziorna (Wrocław Medical University)H-Index: 2
#2Agata Mulak (Wrocław Medical University)H-Index: 14
Background and Aims Bile acids (BA) play an important role in the modulation of numerous gut functions. Fibroblast growth factor 19 (FGF19) is the ileal hormone regulating BA homeostasis. The aim of the study was to evaluate serum FGF19 level and its correlation with clinical and endoscopic disease activity indices along with inflammatory biomarkers including serum CRP and fecal calprotectin levels in patients with ulcerative colitis (UC). Methods Fasting serum FGF19 level was measured using ELI...