Prevalence and long‐term outcome of sub‐clinical primary sclerosing cholangitis in patients with ulcerative colitis

Culver, Emma L.; Bungay, Helen; Betts, Margaret; Forde, Colm; Buchel, Otto C.; Manganis, Charis; Warren, Bryan F.; Cummings, JR Fraser; Keshav, Satish; Travis, Simon; Chapman, Roger W.

doi:10.1111/liv.14645

Cited by 15 publications

(12 citation statements)

References 43 publications

(90 reference statements)

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“…16 This may be attributable to increased awareness and better diagnostics driving recognition of subclinical sclerosing cholangitis in patients with IBD. 31,32 Indeed, data from Norway show that in a cohort of 322 patients with IBD, 17 individuals developed overt biliary lesions on cholangiography despite normal liver biochemistry. 31 Moreover, crosssectional data from the UK found that up to 14% of patients with IBD manifest radiologic features of sclerosing cholangitis in the face of normal alkaline phosphatase values.…”

Section: Discussionmentioning

confidence: 99%

“…31 Moreover, crosssectional data from the UK found that up to 14% of patients with IBD manifest radiologic features of sclerosing cholangitis in the face of normal alkaline phosphatase values. 32 This growing body of data indicates that even the most contemporary of population-based estimates may underestimate the incidence of PSC by a considerable margin.…”

Section: Discussionmentioning

confidence: 99%

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Epidemiology, Natural History, and Outcomes of Primary Sclerosing Cholangitis: A Systematic Review of Population-based Studies

Trivedi

Bowlus

Yimam

et al. 2022

Clinical Gastroenterology and Hepatology

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BACKGROUND & AIMS:The aim of this study was to quantify the global epidemiology of primary sclerosing cholangitis (PSC), alongside the incidence of liver transplantation, cancer, and death, through robust systematic review of population-based data. METHODS:We searched MEDLINE and EMBASE up to and including June 30, 2020 to identify populationbased studies reporting the incidence and/or prevalence of PSC. Studies that did not report original data, or of exclusively pediatric-onset disease (diagnosis age <16 years) or exclusively PSC-associated with inflammatory bowel disease were excluded. RESULTS:Of 4922 published studies, 17 fulfilled inclusion criteria; 16 documenting incidence and 14 prevalence. The highest reported incidence of PSC was reported in Northern Europe (Finland, 1.58 and Norway, 1.3 per-100,000 population, respectively) and North America (Minnesota, 1.47); with the lowest being observed across the Mediterranean Basin (Italy, 0.1). Prevalence ranged from 31.7 in Finland and 23.99 in Minnesota, to 1.33 in Singapore and 0.0 in Alaska. Of studies reporting temporal occurrence, an increase in disease incidence was observed across North America and Northern Europe (4 studies), alongside an increase in prevalence over time (4 studies). The incidence and risks for clinical outcomes were presented by 9 of the included studies. Median transplant-free survival ranged from 9.7 (United States) to 20.6 years (Netherlands), with standardized mortality ratios of 2.5 and 4.2 compared with the control population. The standardized incidence of cholangiocarcinoma ranged from 235 (Finland) to 398 (Netherlands). CONCLUSIONS:Estimates of PSC incidence and prevalence vary, with most studies conducted in North America and Western Europe; the latter showing a steady increase in disease occurrence over time.Further research is needed to understand changes in disease epidemiology, including etiological drivers, the implications of rising case burden on health care policy, and better appreciation of PSC in the developing world.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Epidemiology, Natural History, and Outcomes of Primary Sclerosing Cholangitis: A Systematic Review of Population-based Studies

Trivedi

Bowlus

Yimam

et al. 2022

Clinical Gastroenterology and Hepatology

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“…However, many people with PSC likely remain undiagnosed. [40,41,[152][153][154] Patient demographics and PSC phenotype influence disease progression. Younger age at diagnosis and female sex are associated with better outcomes.…”

Section: Natural History Of Pscmentioning

confidence: 99%

AASLD practice guidance on primary sclerosing cholangitis and cholangiocarcinoma

et al. 2022

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Inclusion of guidance for the diagnosis and management of cholangiocarcinoma (CCA) in patients with and without primary sclerosing cholangitis (PSC) (Figures 5, 8, and 9). Introduction of the term relevant stricture, defined as any biliary stricture of the common hepatic duct or hepatic ducts associated with signs or symptoms of obstructive cholestasis and/or bacterial cholangitis (Table 1). In patients with equivocal MRI with cholangiopancreatography (MRI/MRCP) findings, a repeated high-quality MRI/ MRCP should be performed for diagnostic purposes. Endoscopic retrograde cholangiopancreatography (ERCP) should be avoided for the diagnosis of PSC (Figure 2).

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“…A large population study revealed that patients with long‐standing IBD had a threefold higher prevalence of PSC on magnetic resonance cholangiopancreatography (MRCP) imaging than would have been detected via symptomatic assessment 22 . Furthermore, two studies have shown that biochemical (alkaline phosphatase) levels may not increase over time in those with subclinical PSC 22,23 . As such, these patients may never undergo cross‐sectional screening as a lack of biochemical elevation would classically signal to physicians that PSC has been ruled out.…”

Section: Discussionmentioning

confidence: 99%

The impact of primary sclerosing cholangitis or inflammatory bowel disease on cholangiocarcinoma phenotype, therapy, and survival

2020

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Background and Aim Primary sclerosing cholangitis (PSC), with or without inflammatory bowel disease (IBD), confers the risk of cholangiocarcinoma. Isolated IBD may be an independent risk factor for cholangiocarcinoma. We sought to compare cholangiocarcinoma phenotype and outcomes between patients with PSC, IBD, and neither. Methods Patients with malignancy were separated into cohorts by the presence of PSC and IBD. Data regarding demographics, clinical presentation, therapeutic regimens, and survival were collected. Statistical analysis was carried out using GraphPad and R‐Studio. Results Of 946 patients, 22 had PSC, and 18 had isolated IBD. PSC and IBD patients were younger than controls ( P < 0.001, P = 0.01). Cholangiocarcinoma prevalence was estimated at 0.01% for IBD patients, 0.6% for PSC patients, and 0.002% for all other patients. All cohorts most often presented at stage 4. PSC patients presented more often at stage 3 ( P = 0.04) and with perihilar disease ( P = 0.001). Patients with PSC or IBD received less chemotherapy ( P = 0.004, 0.01). Median overall survivals were 15 months (PSC), 11 months (IBD), and 10 months (controls) ( P = 0.79). Patients with intrahepatic tumors had longer survival ( P < 0.001). Curative intent resection improved survival in all cohorts ( P < 0.001). Multivariate regression identified resection as a predictor of improved survival. Extrahepatic, perihilar, gallbladder, and unspecified biliary tumors were predictors of death. Conclusions Cholangiocarcinoma presents at a late stage and portends dismal survival regardless of PSC or IBD status. Survival was dependent on tumor location and surgical resection. These data suggest that efforts should focus on developing protocols that are able to detect and treat cholangiocarcinoma in high‐risk populations (PSC) at an early stage.

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Prevalence and long‐term outcome of sub‐clinical primary sclerosing cholangitis in patients with ulcerative colitis

Abstract: This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

Cited by 15 publications

References 43 publications

Epidemiology, Natural History, and Outcomes of Primary Sclerosing Cholangitis: A Systematic Review of Population-based Studies

Epidemiology, Natural History, and Outcomes of Primary Sclerosing Cholangitis: A Systematic Review of Population-based Studies

AASLD practice guidance on primary sclerosing cholangitis and cholangiocarcinoma

The impact of primary sclerosing cholangitis or inflammatory bowel disease on cholangiocarcinoma phenotype, therapy, and survival

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