Primary sclerosing cholangitis–inflammatory bowel disease: Epidemiology, mortality, and impact of diagnostic sequence
Background & Aims: Primary sclerosing cholangitis (PSC) carries significant morbidity and mortality compared with inflammatory bowel disease (IBD). We characterized epidemiology trends and outcomes in those with PSC-IBD and IBD, paying particular attention to the impact of PSC-IBD diagnostic...
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Elsevier
2025-03-01
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| author | Kristel K. Leung Wenbin Li Bettina Hansen Aliya Gulamhusein Lauren Lapointe-Shaw Abdel Aziz Shaheen Amanda Ricciuto Eric I. Benchimol Jennifer A. Flemming Gideon M. Hirschfield |
| author_facet | Kristel K. Leung Wenbin Li Bettina Hansen Aliya Gulamhusein Lauren Lapointe-Shaw Abdel Aziz Shaheen Amanda Ricciuto Eric I. Benchimol Jennifer A. Flemming Gideon M. Hirschfield |
| author_sort | Kristel K. Leung |
| collection | DOAJ |
| description | Background & Aims: Primary sclerosing cholangitis (PSC) carries significant morbidity and mortality compared with inflammatory bowel disease (IBD). We characterized epidemiology trends and outcomes in those with PSC-IBD and IBD, paying particular attention to the impact of PSC-IBD diagnostic sequence on outcomes. Methods: Incidence and prevalence of PSC-IBD and IBD (2002–2018) were evaluated using validated health administrative data-derived cohorts from Ontario, Canada (population ∼15 million). Transplant and death outcomes were assessed, with PSC-IBD diagnostic sequence as the exposure of interest. Results: Incidence of PSC-IBD and IBD was 0.46 and 24.6/100,000 person-years (PYs) respectively, whereas prevalence was 5.53 and 588/100,000 PY respectively. Incidence/prevalence of PSC-IBD increased over time, unlike for IBD. Age at IBD diagnosis was earlier among those with PSC-IBD compared with those with IBD alone. Higher socioeconomic status associated with high PSC-IBD incidence rates and fastest incidence rise. Those diagnosed with IBD before PSC had higher risk of transplant/death compared with PSC before IBD (hazard ratio [HR] 1.34, 95% CI 1.02–1.75), driven by an increased risk of death (HR 2.73, 95% CI 1.68–4.45). PSC-IBD had a 4.5-fold greater risk of transplant/death compared with IBD alone. Liver-related and luminal gastrointestinal disease, particularly hepatopancreatobiliary malignancy, were predominant causes of death among those with PSC-IBD, while cardiovascular and respiratory diseases were predominant among those with IBD. Conclusions: Population-level data support distinct epidemiological patterns among people living with PSC-IBD compared with IBD, including a higher socioeconomic status and worse outcomes in those found to have IBD before PSC. Impact and implications:: Individuals with primary sclerosing cholangitis (PSC) face increased morbidity and mortality compared with the general population and those with inflammatory bowel disease (IBD); yet, most individuals with PSC are found to have concomitant IBD during their lifetime. This study describes the distinctive epidemiological differences and mortality trends at the population level between PSC-IBD and IBD. While PSC-IBD remains a rare condition, diagnoses are on the rise (particularly among higher socioeconomic status populations), with most patients being diagnosed with IBD before PSC; this group also experienced higher mortality post-PSC diagnosis compared with those diagnosed with PSC first, with a large proportion of deaths caused by liver- and gut-related causes. Practical applications of these findings include further studies to evaluate whether earlier identification of PSC-IBD affects disease outcomes, as well as educating patients, clinicians, and policymakers on the importance of recognizing PSC-IBD as a distinct entity from IBD alone. |
| format | Article |
| id | doaj-art-b8739fa7aa2b4210a27952041b0a5fdd |
| institution | DOAJ |
| issn | 2589-5559 |
| language | English |
| publishDate | 2025-03-01 |
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| spelling | doaj-art-b8739fa7aa2b4210a27952041b0a5fdd2025-08-20T02:43:39ZengElsevierJHEP Reports2589-55592025-03-017310127210.1016/j.jhepr.2024.101272Primary sclerosing cholangitis–inflammatory bowel disease: Epidemiology, mortality, and impact of diagnostic sequenceKristel K. Leung0Wenbin Li1Bettina Hansen2Aliya Gulamhusein3Lauren Lapointe-Shaw4Abdel Aziz Shaheen5Amanda Ricciuto6Eric I. Benchimol7Jennifer A. Flemming8Gideon M. Hirschfield9The Autoimmune and Rare Liver Disease Programme, Division of Gastroenterology and Hepatology, Toronto General Hospital, Toronto, ON, Canada; Institute of Health Policy, Management, and Evaluation, Dalla Lana School of Public Health, University of Toronto, Toronto, ON, Canada; Departments of Medicine & Public Health Sciences, Queen's University, Kingston, ON, CanadaICES-Queen's, Kingston, ON, CanadaThe Autoimmune and Rare Liver Disease Programme, Division of Gastroenterology and Hepatology, Toronto General Hospital, Toronto, ON, Canada; Institute of Health Policy, Management, and Evaluation, Dalla Lana School of Public Health, University of Toronto, Toronto, ON, Canada; Department of Biostatistics & Epidemiology, Erasmus MC University Medical Center, Rotterdam, The NetherlandsThe Autoimmune and Rare Liver Disease Programme, Division of Gastroenterology and Hepatology, Toronto General Hospital, Toronto, ON, Canada; Institute of Health Policy, Management, and Evaluation, Dalla Lana School of Public Health, University of Toronto, Toronto, ON, CanadaInstitute of Health Policy, Management, and Evaluation, Dalla Lana School of Public Health, University of Toronto, Toronto, ON, Canada; ICES, Toronto, ON, Canada; Women's College Institute for Health System Solutions and Virtual Care, Women's College Hospital, Division of General Internal Medicine and Geriatrics, University Health Network and Sinai Health System, Toronto, ON, CanadaGastroenterology, Department of Medicine and Community Health Sciences, University of Calgary, Calgary, AB, CanadaSickKids Inflammatory Bowel Disease Centre, Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, Toronto, ON, Canada; Department of Paediatrics, University of Toronto, Toronto, ON, CanadaInstitute of Health Policy, Management, and Evaluation, Dalla Lana School of Public Health, University of Toronto, Toronto, ON, Canada; ICES, Toronto, ON, Canada; SickKids Inflammatory Bowel Disease Centre, Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, Toronto, ON, Canada; Department of Paediatrics, University of Toronto, Toronto, ON, Canada; Child Health Evaluative Sciences, SickKids Research Institute, The Hospital for Sick Children, Toronto, ON, CanadaDepartments of Medicine & Public Health Sciences, Queen's University, Kingston, ON, Canada; ICES-Queen's, Kingston, ON, CanadaThe Autoimmune and Rare Liver Disease Programme, Division of Gastroenterology and Hepatology, Toronto General Hospital, Toronto, ON, Canada; Institute of Health Policy, Management, and Evaluation, Dalla Lana School of Public Health, University of Toronto, Toronto, ON, Canada; Corresponding author. Address: The Autoimmune and Rare Liver Disease Programme, Division of Gastroenterology and Hepatology, Toronto General Hospital, 200 Elizabeth St., Toronto, ON, M5G 2C4, Canada.Background & Aims: Primary sclerosing cholangitis (PSC) carries significant morbidity and mortality compared with inflammatory bowel disease (IBD). We characterized epidemiology trends and outcomes in those with PSC-IBD and IBD, paying particular attention to the impact of PSC-IBD diagnostic sequence on outcomes. Methods: Incidence and prevalence of PSC-IBD and IBD (2002–2018) were evaluated using validated health administrative data-derived cohorts from Ontario, Canada (population ∼15 million). Transplant and death outcomes were assessed, with PSC-IBD diagnostic sequence as the exposure of interest. Results: Incidence of PSC-IBD and IBD was 0.46 and 24.6/100,000 person-years (PYs) respectively, whereas prevalence was 5.53 and 588/100,000 PY respectively. Incidence/prevalence of PSC-IBD increased over time, unlike for IBD. Age at IBD diagnosis was earlier among those with PSC-IBD compared with those with IBD alone. Higher socioeconomic status associated with high PSC-IBD incidence rates and fastest incidence rise. Those diagnosed with IBD before PSC had higher risk of transplant/death compared with PSC before IBD (hazard ratio [HR] 1.34, 95% CI 1.02–1.75), driven by an increased risk of death (HR 2.73, 95% CI 1.68–4.45). PSC-IBD had a 4.5-fold greater risk of transplant/death compared with IBD alone. Liver-related and luminal gastrointestinal disease, particularly hepatopancreatobiliary malignancy, were predominant causes of death among those with PSC-IBD, while cardiovascular and respiratory diseases were predominant among those with IBD. Conclusions: Population-level data support distinct epidemiological patterns among people living with PSC-IBD compared with IBD, including a higher socioeconomic status and worse outcomes in those found to have IBD before PSC. Impact and implications:: Individuals with primary sclerosing cholangitis (PSC) face increased morbidity and mortality compared with the general population and those with inflammatory bowel disease (IBD); yet, most individuals with PSC are found to have concomitant IBD during their lifetime. This study describes the distinctive epidemiological differences and mortality trends at the population level between PSC-IBD and IBD. While PSC-IBD remains a rare condition, diagnoses are on the rise (particularly among higher socioeconomic status populations), with most patients being diagnosed with IBD before PSC; this group also experienced higher mortality post-PSC diagnosis compared with those diagnosed with PSC first, with a large proportion of deaths caused by liver- and gut-related causes. Practical applications of these findings include further studies to evaluate whether earlier identification of PSC-IBD affects disease outcomes, as well as educating patients, clinicians, and policymakers on the importance of recognizing PSC-IBD as a distinct entity from IBD alone.http://www.sciencedirect.com/science/article/pii/S2589555924002763EpidemiologyOutcomesHealth administrative data |
| spellingShingle | Kristel K. Leung Wenbin Li Bettina Hansen Aliya Gulamhusein Lauren Lapointe-Shaw Abdel Aziz Shaheen Amanda Ricciuto Eric I. Benchimol Jennifer A. Flemming Gideon M. Hirschfield Primary sclerosing cholangitis–inflammatory bowel disease: Epidemiology, mortality, and impact of diagnostic sequence JHEP Reports Epidemiology Outcomes Health administrative data |
| title | Primary sclerosing cholangitis–inflammatory bowel disease: Epidemiology, mortality, and impact of diagnostic sequence |
| title_full | Primary sclerosing cholangitis–inflammatory bowel disease: Epidemiology, mortality, and impact of diagnostic sequence |
| title_fullStr | Primary sclerosing cholangitis–inflammatory bowel disease: Epidemiology, mortality, and impact of diagnostic sequence |
| title_full_unstemmed | Primary sclerosing cholangitis–inflammatory bowel disease: Epidemiology, mortality, and impact of diagnostic sequence |
| title_short | Primary sclerosing cholangitis–inflammatory bowel disease: Epidemiology, mortality, and impact of diagnostic sequence |
| title_sort | primary sclerosing cholangitis inflammatory bowel disease epidemiology mortality and impact of diagnostic sequence |
| topic | Epidemiology Outcomes Health administrative data |
| url | http://www.sciencedirect.com/science/article/pii/S2589555924002763 |
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