Primary sclerosing cholangitis, autoimmune hepatitis, and overlap in Utah children: epidemiology and natural history
Open accessAbstract
The epidemiology and natural history of pediatric primary sclerosing cholangitis (PSC), autoimmune sclerosing cholangitis (ASC), and autoimmune hepatitis (AIH) are not well characterized. Using multiple, overlapping search strategies followed by a detailed records review, we identified all cases of pediatric PSC, ASC, AIH, and inflammatory bowel disease (IBD) in a geographically isolated region of the United States. We identified 607 cases of IBD, 29 cases of PSC, 12 cases of ASC, and 44 cases of AIH. The mean age at diagnosis was 13.0 years for PSC, 11.3 years for ASC, and 9.8 years for AIH. The incidence and prevalence of PSC, ASC, and AIH were 0.2 and 1.5 cases, 0.1 and 0.6 cases, and 0.4 and 3.0 cases per 100,000 children, respectively. The mean duration of follow-up was 5.9 years. The probability of developing complicated liver disease within 5 years of the diagnosis of liver disease was 37% [95% confidence interval (CI) = 21%-58%] for PSC, 25% (95% CI = 7%-70%) for ASC, and 15% (95% CI = 7%-33%) for AIH. The 5-year survival rates with the native liver were 78% (95% CI = 54%-91%) for PSC, 90% (95% CI = 47%-99%) for ASC, and 87% (95% CI = 71%-95%) for AIH. Cholangiocarcinoma developed in 2 of the 29 PSC patients (6.9%). PSC occurred in 9.9% of patients with ulcerative colitis (UC) and in 0.6% of patients with Crohn's disease (CD). ASC occurred in 2.3% of UC patients and 0.9% of CD patients. AIH occurred in 0.4% of UC patients and in 0.3% of CD patients. Liver disease occurred in 39 of 607 IBD patients (6.4%) overall. Conclusion : Immune-mediated liver diseases are important sources of morbidity in children. Using a population-based design, this study quantifies the burden and natural history of immune-mediated liver disease in children. (Hepatology 2013;58:1392–1400)
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A population-based multicentre study in the USA estimated the prevalence of PSC as 0.2/10,000 children compared to up to 16/100,000 in adults.
“A population-based multicentre study in the USA estimated the prevalence of PSC as 0.2/10,000 children compared to up to 16/100,000 in adults.424”
From EASL Clinical Practice Guidelines on sclerosing cholangitis (2022) · cites this paper as [424]
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Cites
Simplified criteria for the diagnosis of autoimmune hepatitis
Hennes, E. M., Zeniya, M., Czaja, A. J., Parés, A., Dalekos, G. N., Krawitt, E. L., Bittencourt, P. L., Porta, G., Boberg, K. M., Hofer, H., et al. (2008) · Hepatology · doi:10.1002/hep.22322 · Cited by 1,901 · see citing works
Diagnosis and management of autoimmune hepatitis
Manns, M. P., Czaja, A. J., Gorham, J. D., Krawitt, E. L., Mieli-Vergani, G., Vergani, D., & Vierling, J. M. (2010) · Hepatology · doi:10.1002/hep.23584 · Cited by 1,442 · see citing works
Diagnosis and Management of Primary Sclerosing Cholangitis
Chapman, R. W., Fevery, J., Kalloo, A. N., Nagorney, D. M., Boberg, K. M., Shneider, B. L., & Gores, G. J. (2009) · Hepatology · doi:10.1002/hep.23294 · Cited by 1,217 · see citing works
Natural history and prognostic factors in 305 Swedish patients with primary sclerosing cholangitis.
Broomé, U., Olsson, R., Lööf, L., Bodemar, G., Hultcrantz, R., Danielsson, A., Prytz, H., Sandberg–Gertzén, H., Wallerstedt, S. M., & Lindberg, G. (1996) · Gut · doi:10.1136/gut.38.4.610 · Cited by 816 · see citing works
The Prevalence of Extraintestinal Diseases in Inflammatory Bowel Disease: A Population-Based Study
Bernstein, C. N., Blanchard, J., Rawsthorne, P. E., & Yu, N. (2001) · The American Journal of Gastroenterology · doi:10.1111/j.1572-0241.2001.03756.x · Cited by 816 · see citing works
Gregorio, G. (2001) · Hepatology · doi:10.1053/jhep.2001.22131 · Cited by 692 · see citing works
Autoimmune Hepatitis in Childhood: A 20–Year Experience
Gregorio, G. V., Portmann, B. C., Reid, F., Donaldson, P. T., Doherty, D. G., McCartney, M., Mowat, A. P., Vergani, D., & Mieli‐Vergani, G. (1997) · Hepatology · doi:10.1002/hep.510250308 · Cited by 662 · see citing works
Epidemiology of primary sclerosing cholangitis and primary biliary cirrhosis: A systematic review
Boonstra, K., Beuers, U., & Ponsioen, C. Y. (2012) · Journal of Hepatology · doi:10.1016/j.jhep.2011.10.025 · Cited by 632 · see citing works
Vavricka, S. R., Brun, L., Ballabeni, P., et al. (2010) · The American Journal of Gastroenterology · doi:10.1038/ajg.2010.343 · Cited by 598 · see citing works
Consensus Conference: Colorectal Cancer Screening and Surveillance in Inflammatory Bowel Disease
Itzkowitz, S. H., & Present, D. H. (2005) · Inflammatory Bowel Diseases · doi:10.1097/01.mib.0000160811.76729.d5 · Cited by 570 · see citing works
Boberg, K. M., Chapman, R. W., Hirschfield, G. M., Lohse, A. W., Manns, M. P., & Schrumpf, E. (2011) · Journal of Hepatology · doi:10.1016/j.jhep.2010.09.002 · Cited by 555 · see citing works
Farraye, F. A., Odze, R. D., Eaden, J., & Itzkowitz, S. H. (2010) · Gastroenterology · doi:10.1053/j.gastro.2009.12.037 · Cited by 530 · see citing works
Decreasing Risk of Colorectal Cancer in Patients With Inflammatory Bowel Disease Over 30 Years
Jess, T., Simonsen, J., Jørgensen, K. T., Pedersen, B. V., Nielsen, N. M., & Frisch, M. (2012) · Gastroenterology · doi:10.1053/j.gastro.2012.04.016 · Cited by 530 · see citing works
Boberg, K. M. (1998) · Scandinavian Journal of Gastroenterology · doi:10.1080/00365529850166284 · Cited by 514 · see citing works
Bambha, K. M., Kim, W. R., Talwalkar, J. A., Torgerson, H., Benson, J. T., Therneau, T. M., Loftus, E. V., Yawn, B. P., Dickson, E. R., & Melton, L. J. (2003) · Gastroenterology · doi:10.1016/j.gastro.2003.07.011 · Cited by 450 · see citing works
Prevalence of Primary Sclerosing Cholangitis in Patients With Ulcerative Colitis
Olsson, R., Danielsson, Å., Järnerot, G., Lindström, E., Lööf, L., Rolny, P., Rydén, B.-O., Tysk, C., & Wallerstedt, S. (1991) · Gastroenterology · doi:10.1016/0016-5085(91)70019-t · Cited by 424 · see citing works
Nguyen, G. C., Torres, E. A., Regueiro, M. D., Bromfield, G. P., Bitton, A., Stempak, J. M., Dassopoulos, T., Schumm, L. P., Gregory, F. J., Griffiths, A. M., et al. (2006) · The American Journal of Gastroenterology · doi:10.1111/j.1572-0241.2006.00504.x · Cited by 285 · see citing works
Kaplan, G. G., Laupland, K. B., Butzner, D., Urbanski, S. J., & Lee, S. S. (2007) · The American Journal of Gastroenterology · doi:10.1111/j.1572-0241.2007.01103.x · Cited by 273 · see citing works
Ponsioen, C. Y., Vrouenraets, S. M. E., Prawirodirdjo, W., Rajaram, R., Rauws, E. A., Mulder, C. J. J., Reitsma, J. B., Heisterkamp, S. H., & Tytgat, G. N. J. (2002) · Gut · doi:10.1136/gut.51.4.562 · Cited by 267 · see citing works
Prevalence of autoimmune liver disease in Alaska natives
Hurlburt, K. J., McMahon, B. J., Deubner, H., Hsu-Trawinski, B., Williams, J. L., & Kowdley, K. V. (2002) · The American Journal of Gastroenterology · doi:10.1111/j.1572-0241.2002.06019.x · Cited by 263 · see citing works
Primary Sclerosing Cholangitis in Children: A Long–Term Follow–Up Study
Feldstein, A. E., Perrault, J. F., El-Youssif, M., Lindor, K. D., Freese, D. K., & Angulo, P. (2003) · Hepatology · doi:10.1053/jhep.2003.50289 · Cited by 251 · see citing works
Primary sclerosing cholangitis: Summary of a workshop
LaRusso, N. F., Shneider, B. L., Black, D. D., Gores, G. J., James, S. P., Doo, E. C., & Hoofnagle, J. H. (2006) · Hepatology · doi:10.1002/hep.21337 · Cited by 248 · see citing works
Dotson, J. L., Hyams, J. S., Markowitz, J. F., LeLeiko, N. S., Mack, D. R., Evans, J., Pfefferkorn, M. D., Griffiths, A. M., Otley, A. R., Bousvaros, A., et al. (2010) · Journal of Pediatric Gastroenterology and Nutrition · doi:10.1097/mpg.0b013e3181ca4db4 · Cited by 200 · see citing works
Treatment options for autoimmune hepatitis: A systematic review of randomized controlled trials
Lamers, M. H., van Oijen, M. G. H., Pronk, M., & Drenth, J. P. H. (2010) · Journal of Hepatology · doi:10.1016/j.jhep.2010.01.037 · Cited by 184 · see citing works
Population‐based epidemiology study of autoimmune hepatitis: A disease of older women?
Ngu, J. H., Bechly, K., CHAPMAN, B. A., Burt, M. J., Barclay, M. L., Gearry, R. B., & Stedman, C. A. M. (2010) · Journal of Gastroenterology and Hepatology · doi:10.1111/j.1440-1746.2010.06384.x · Cited by 181 · see citing works
A Retrospective Single-Center Review of Primary Sclerosing Cholangitis in Children
Miloh, T., Arnon, R., Shneider, B. L., Suchy, F. J., & Kerkar, N. N. (2008) · Clinical Gastroenterology and Hepatology · doi:10.1016/j.cgh.2008.10.019 · Cited by 150 · see citing works
Surgical pathology of the syndrome of primary sclerosing cholangitis.
Ludwig, J. (1989) · PubMed · Cited by 113 · see citing works
Fine-scaled human genetic structure revealed by SNP microarraysPreprint
Xing, J., Watkins, W. S., Witherspoon, D. J., Zhang, Y., Guthery, S. L., THARA, R., Mowry, B. J., Bulayeva, K., Weiss, R. B., & Jorde, L. B. (2009) · Genome Research · doi:10.1101/gr.085589.108 · Cited by 93 · see citing works
Self-citationValidation and Modification of Simplified Diagnostic Criteria for Autoimmune Hepatitis in Children
Mileti, E., Rosenthal, P. J., & Peters, M. G. (2011) · Clinical Gastroenterology and Hepatology · doi:10.1016/j.cgh.2011.11.030 · Cited by 90 · see citing works
Clinical Characteristics of Inflammatory Bowel Disease in Turkey
Tozun, N. A., Atuğ, Ö., İmeryüz, N., Hamzaoğlu, H. Ö., Tiftikçi, A., Parlak, E., Dağlı, Ü., Ülker, A., Hülagü, S., Akpınar, H., et al. (2008) · Journal of Clinical Gastroenterology · doi:10.1097/mcg.0b013e3181574636 · Cited by 77 · see citing works
The Overlap Syndromes of Autoimmune Hepatitis
Czaja, A. J. (2012) · Digestive Diseases and Sciences · doi:10.1007/s10620-012-2367-1 · Cited by 76 · see citing works
Radhakrishnan, K. R., Alkhouri, N., Worley, S. E., Arrigain, S., Hupertz, V. F., Kay, M. H., Yerian, L. M., Wyllie, R., & Feldstein, A. E. (2010) · Digestive and Liver Disease · doi:10.1016/j.dld.2010.01.002 · Cited by 68 · see citing works
Lepistö, A., Kärkkäinen, P. A., & Järvinen, H. (2008) · Inflammatory Bowel Diseases · doi:10.1002/ibd.20384 · Cited by 56 · see citing works
Dassopoulos, T., Nguyen, G. C., Bitton, A. M., et al. (2007) · Inflammatory Bowel Diseases · doi:10.1002/ibd.20144 · Cited by 45 · see citing works
Heikius, B., Niemelä, S., Lehtola, J., Karttunen, T. J., & Lahde, S. (1997) · Scandinavian Journal of Gastroenterology · doi:10.3109/00365529709000186 · Cited by 45 · see citing works
Autoimmune Hepatitis in Children in Eastern Denmark
Vitfell-Pedersen, J., Jørgensen, M. H., Müller, K., & Heilmann, C. J. (2012) · Journal of Pediatric Gastroenterology and Nutrition · doi:10.1097/mpg.0b013e3182602b20 · Cited by 43 · see citing works
HISTOPATHOLOGY OF CHRONIC CHOLESTASIS AND ADULT DUCTOPENIC SYNDROME
Desmet, V. J. (1998) · Clinics in Liver Disease · doi:10.1016/s1089-3261(05)70006-4 · Cited by 31 · see citing works
Chung, H. V., Riley, M., Ho, J. K., Leung, B. K., Jevon, G. P., Arbour, L. T., Barker, C., Schreiber, R. A., & Yoshida, E. M. (2007) · Canadian Journal of Gastroenterology · doi:10.1155/2007/757906 · Cited by 29 · see citing works
Alexopoulou, E., Xenophontos, P. E., Economopoulos, N., Spyridopoulos, T. N., Papakonstantinou, O., Panayotou, I., Dimakou, K., Roma, E. S., & Kelekis, N. (2012) · Journal of Pediatric Gastroenterology and Nutrition · doi:10.1097/mpg.0b013e31825bb3dc · Cited by 29 · see citing works
Lindberg, J., Stenling, R., Palmqvist, R., et al. (2008) · Journal of Pediatric Gastroenterology and Nutrition · doi:10.1097/mpg.0b013e31815a98ef · Cited by 27 · see citing works
Characterization of Hepatic Abnormalities in Children with Inflammatory Bowel Disease
Hyams, J. S., Markowitz, J. F., Treem, W. R., et al. (1995) · Inflammatory Bowel Diseases · doi:10.1097/00054725-199503000-00003 · Cited by 24 · see citing works
Characterization of hepatic abnormalities in children with inflammatory bowel disease
Hyams, J. S., Markowitz, J. F., Treem, W. R., et al. (1995) · Inflammatory Bowel Diseases · doi:10.1002/ibd.3780010104 · Cited by 23 · see citing works
Deneau, M. R., Adler, D. G., Schwartz, J. J., et al. (2011) · Journal of Pediatric Gastroenterology and Nutrition · doi:10.1097/mpg.0b013e3181f9a5d2 · Cited by 22 · see citing works
Self-citationNguyen, G. C., & Bressler, B. L. (2012) · Gastroenterology · doi:10.1053/j.gastro.2012.06.027 · Cited by 17 · see citing works
Sclerosing cholangitis in children with inflammatory bowel disease
Ong, J. C., O'Loughlin, E. V., Kamath, K. R., Dorney, S. F. A., SILVA, M., & Gaskin, K. J. (1994) · Australian and New Zealand Journal of Medicine · doi:10.1111/j.1445-5994.1994.tb00550.x · Cited by 14 · see citing works
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Research works printed but not linked: 1
- Jimenez-Rivera C, Graitson S, Critch J, Rashid M, Ling S, Ng V, et al. Incidence and characteristics of autoimmune hepatitis in children and adolescents in Canada: a preliminary report of the Canadian Pediatric Hepatology Research Group [abstract]. HEPATOLOGY 2012;54:138A.
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Karlsen, T. H., Folseraas, T., Thorburn, D., & Vesterhus, M. (2017) · Journal of Hepatology · doi:10.1016/j.jhep.2017.07.022 · Cited by 868 · see citing works
EASL Clinical Practice Guidelines on sclerosing cholangitis
Chazouilleres, O., Beuers, U., Bergquist, A., Karlsen, T. H., Levy, C., Samyn, M., Schramm, C., & Trauner, M. (2022) · Journal of Hepatology · doi:10.1016/j.jhep.2022.05.011 · Cited by 419 · see citing works
Chapman, M. H., Thorburn, D., Hirschfield, G. M., Webster, G. G. J., Rushbrook, S. M., Alexander, G., Collier, J., Dyson, J. K., Jones, D. E., Patanwala, I., et al. (2019) · Gut · doi:10.1136/gutjnl-2018-317993 · Cited by 284 · see citing works
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- Authors
- Deneau, M., Jensen, K. M., Holmen, J., Williams, M. S., Book, L. S., & Guthery, S. L.
- Affiliations
- University of Utah (US)
- Intermountain Healthcare (US)
- Geisinger Health System (US)
- Corresponding author
- Deneau, Mark
- Venue
- Hepatology
- Published
- 2013-10
- Volume
- 58
- Issue
- 4
- Pages
- 1392-1400
- ISSN
- 0270-9139 (ISSN-L)
- OpenAlex
- W1507163675
- Cited by
- 233
- References
- 45
- Citations by year
- 2026: 15 · 2025: 14 · 2024: 13 · 2023: 16 · 2022: 20 · 2021: 26 · 2020: 14 · 2019: 28 · 2018: 20 · 2017: 22 · 2016: 16 · 2015: 16 · 2014: 10 · 2013: 1 · 2012: 1
- FWCI
- 8.94
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Hepatology (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institutes of Health (UL1TR000077, UL1TR000105)
- National Center for Research Resources (UL1RR025764)
- National Center for Advancing Translational Sciences
- Topics
- Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
- Health Sciences › Medicine › Surgery › Pediatric Hepatobiliary Diseases and Treatments
- Health Sciences › Medicine › Rheumatology › IgG4-Related and Inflammatory Diseases
- Keywords
- primary sclerosing cholangitis, autoimmune sclerosing cholangitis, autoimmune hepatitis, pediatric liver disease, inflammatory bowel disease, ulcerative colitis, Crohn's disease, cholangiocarcinoma, incidence and prevalence, natural history (disease), survival, immune-mediated liver disease, disease overlap, epidemiology
- MeSH terms
- Adolescent, Autoimmune Diseases, Child, Child, Preschool, Female, Humans, Infant, Male, Retrospective Studies, Utah, Cholangitis, Sclerosing, Prevalence, Survival Rate, Hepatitis, Autoimmune
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