Prevalence of Primary Sclerosing Cholangitis in Patients With Inflammatory Bowel Disease: A Systematic Review and Meta-analysis

Barberio, Brigida; Massimi, Davide; Cazzagon, Nora; Zingone, Fabiana; Ford, Alexander C; Savarino, Edoardo

doi:10.1053/j.gastro.2021.08.032

Cited by 69 publications

(50 citation statements)

References 80 publications

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“…5 Small duct PSC was more common in the setting of CD than UC. 10 In our study, we demonstrated that patients with large duct PSC had a higher prevalence of IBD and UC than patients with small duct PSC. Although there was no significant difference in the prevalence of CD, it seemed that the prevalence of CD was higher in patients with small duct PSC (13.5%, 39/288) compared with large duct PSC (11.0%, 779/7073).…”

Section: Discussionsupporting

confidence: 49%

“…41 A previous meta-analysis had provided the pooled estimates of the burden of PSC in IBD. 10 However, the disease burden of IBD was not well estimated in PSC patients before.…”

Section: Discussionmentioning

confidence: 99%

“…[7][8][9] The association between PSC and IBD has been widely studied, 1 the pooled prevalence of PSC in IBD was 2.2% and was highest in South America and lowest in Southeast Asia. 10 The estimated prevalence of IBD in patients with PSC varies greatly, ranging from 20% to 88%. [11][12][13] However, there was no meta-analysis performed to estimate the pooled prevalence rates.…”

Section: Introductionmentioning

confidence: 99%

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Prevalence of inflammatory bowel disease in patients with primary sclerosing cholangitis: A systematic review and meta‐analysis

Zhang

Gao²,

et al. 2022

Liver International

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Background and Aim: Previous studies have established an association between primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD), including Crohn's disease and ulcerative colitis (UC). The disease burden of IBD in PSC patients was not well estimated. The study aimed to quantify the pooled prevalence of IBD in PSC and to investigate whether subtypes of PSC and sex influence the prevalence of IBD.Methods: PubMed, Embase, and Web of Science were searched through November 2021 for studies reporting data on IBD among PSC patients. The outcomes were the prevalence of IBD in patients with PSC, as well as the association (odds ratio [OR]) of IBD in PSC according to subtype and sex. Results:Based on the analysis of 25 studies, the prevalence of IBD in patients with PSC was 71.1% (95% CI 68.2-75.1%), most commonly in UC (55.9%, 95% CI 52.5-59.3%). The pooled prevalence of IBD was 76.9% in Australia (95% CI 71.2-82.6%, 1 study), 75.9% (95% CI 69.5-82.3%, 4 studies) in North America, 70.9% (95% CI 65.8-76.0%, 17 studies) in Europe and 67.0% (95% CI 57.9-76.0%, 2 studies) in Asia. Male PSC patients had a higher prevalence of IBD (OR 1.67, 95% CI 1.52-1.83) and UC (OR 2.02, 95% CI 1.56-2.63) and a lower prevalence of CD (OR 0.77, 95% CI 0.67-0.88) than female patients. Large duct PSC patients had a higher prevalence of IBD (OR 2.57, 95% CI 2.03-3.25) and UC (OR 4.51,) than small duct PSC patients.

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Section: Discussionsupporting

confidence: 49%

“…41 A previous meta-analysis had provided the pooled estimates of the burden of PSC in IBD. 10 However, the disease burden of IBD was not well estimated in PSC patients before.…”

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Prevalence of inflammatory bowel disease in patients with primary sclerosing cholangitis: A systematic review and meta‐analysis

Zhang

Gao²,

et al. 2022

Liver International

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show abstract

Section: Introductionmentioning

confidence: 99%

“…Although the exact prevalence is unclear, approximately 2%–5% of patients with IBD develop PSC, a chronic disorder characterized by fibrotic biliary strictures, progressing to liver cirrhosis. 14 About 60%–80% of PSC patients have concomitant IBD. 15 PSC might be an independent risk factor for developing gastrointestinal malignancies and it has become increasingly clear that IBD co‐existing with PSC may be distinct from IBD alone, 16 though the aetiology and pathogenesis of PSC remain unclear.…”

Section: Introductionmentioning

confidence: 99%

Risk of hepato‐pancreato‐biliary cancer is increased by primary sclerosing cholangitis in patients with inflammatory bowel disease: A population‐based cohort study

Refsum

Helsingen

et al. 2022

UEG Journal

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Background There is continued uncertainty regarding the risks of hepato‐pancreato‐biliary cancers in patients with inflammatory bowel disease (IBD) with or without concomitant primary sclerosing cholangitis (PSC). Objective To give updated estimates on risk of hepato‐pancreato‐biliary cancers in patients with IBD, including pancreatic cancer, hepatocellular carcinoma, gall bladder cancer, and intra – and extrahepatic cholangiocarcinoma. Methods In a population‐based cohort study, we included all patients diagnosed with IBD in Norway and Sweden from 1987 to 2016. The cohort comprised of 141,960 patients, identified through hospital databases and the National Patient Register. Participants were followed through linkage to national cancer, cause of death, and population registries. We calculated absolute risk and standardized incidence ratios (SIRs) of hepato‐pancreato‐biliary cancers by PSC and other clinical characteristics. Results Of the 141,960 IBD patients, 3.2% were diagnosed with PSC. During a median follow‐up of 10.0 years, we identified 443 biliary tract cancers (SIR 5.2, 95% confidence interval [CI] 4.8–5.7), 161 hepatocellular carcinomas (SIR 2.4, 95% CI 2.0–2.7) and 282 pancreatic cancers (SIR 1.3, 95% CI 1.2–1.5). The relative risks were considerably higher in PSC‐IBD patients, with SIR of 140 (95% CI 123–159) for biliary tract, 38.6 (95% CI 29.2–50.0) for hepatocellular, and 9.0 (95% CI 6.3–12.6) for pancreatic cancer. The SIRs were still slightly increased in non‐PSC‐IBD patients, compared to the general population. For biliary tract cancer, the cumulative probability at 25 years was 15.6% in PSC‐IBD patients, and 0.4% in non‐PSC‐IBD patients. Conclusions The dramatically increased risks of hepato‐pancreato‐biliary cancers in PSC‐IBD patients support periodic surveillance for these malignancies. While much lower, the excess relative risks in non‐PSC‐IBD patients were not trivial compared to non‐IBD related risk factors.

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Ulcerative Colitis in Adults

Gros,

Kaplan

2023

JAMA

144

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ImportanceUlcerative colitis (UC) is a chronic inflammatory condition of the colon, with a prevalence exceeding 400 per 100 000 in North America. Individuals with UC have a lower life expectancy and are at increased risk for colectomy and colorectal cancer.ObservationsUC impairs quality of life secondary to inflammation of the colon causing chronic diarrhea and rectal bleeding. Extraintestinal manifestations, such as primary sclerosing cholangitis, occur in approximately 27% of patients with UC. People with UC require monitoring of symptoms and biomarkers of inflammation (eg, fecal calprotectin), and require colonoscopy at 8 years from diagnosis for surveillance of dysplasia. Risk stratification by disease location (eg, Montreal Classification) and disease activity (eg, Mayo Score) can guide management of UC. First-line therapy for induction and maintenance of remission of mild to moderate UC is 5-aminosalicylic acid. Moderate to severe UC may require oral corticosteroids for induction of remission as a bridge to medications that sustain remission (biologic monoclonal antibodies against tumor necrosis factor [eg, infliximab], α4β7 integrins [vedolizumab], and interleukin [IL] 12 and IL-23 [ustekinumab]) and oral small molecules that inhibit janus kinase (eg, tofacitinib) or modulate sphingosine-1-phosphate (ozanimod). Despite advances in medical therapies, the highest response to these treatments ranges from 30% to 60% in clinical trials. Within 5 years of diagnosis, approximately 20% of patients with UC are hospitalized and approximately 7% undergo colectomy. The risk of colorectal cancer after 20 years of disease duration is 4.5%, and people with UC have a 1.7-fold higher risk for colorectal cancer compared with the general population. Life expectancy in people with UC is approximately 80.5 years for females and 76.7 years for males, which is approximately 5 years shorter than people without UC.Conclusions and RelevanceUC affects approximately 400 of every 100 000 people in North America. An effective treatment for mild to moderate UC is 5-aminosalicylic acid, whereas moderate to severe UC can be treated with advanced therapies that target specific inflammation pathways, including monoclonal antibodies to tumor necrosis factor, α4β7 integrins, and IL-12 and IL-23 cytokines, as well as oral small molecule therapies targeting janus kinase or sphingosine-1-phosphate.

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Prevalence of Primary Sclerosing Cholangitis in Patients With Inflammatory Bowel Disease: A Systematic Review and Meta-analysis

Cited by 69 publications

References 80 publications

Prevalence of inflammatory bowel disease in patients with primary sclerosing cholangitis: A systematic review and meta‐analysis

Prevalence of inflammatory bowel disease in patients with primary sclerosing cholangitis: A systematic review and meta‐analysis

Risk of hepato‐pancreato‐biliary cancer is increased by primary sclerosing cholangitis in patients with inflammatory bowel disease: A population‐based cohort study

Ulcerative Colitis in Adults

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