Primary sclerosing cholangitis, autoimmune hepatitis, and overlap in Utah children: epidemiology and natural history
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Published on: May 29, 2020
Mark Deneau1, M Kyle Jensen, John Holmen
1Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, University of Utah, Salt Lake City, UT.
Insights
This study quantifies pediatric immune-mediated liver diseases like primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH). It reveals disease burden and natural history in children, highlighting significant morbidity.
Area of Science:
- Pediatric Gastroenterology and Hepatology
- Immunology
- Epidemiology
Background:
- The epidemiology and natural history of pediatric primary sclerosing cholangitis (PSC), autoimmune sclerosing cholangitis (ASC), and autoimmune hepatitis (AIH) are not well characterized.
- Immune-mediated liver diseases (IMLDs) contribute significantly to childhood morbidity.
- Understanding the incidence, prevalence, and outcomes of these conditions is crucial for effective management.
Purpose of the Study:
- To characterize the epidemiology and natural history of pediatric PSC, ASC, and AIH.
- To quantify the burden of these diseases in a geographically isolated population.
- To assess the long-term outcomes, including complications and survival rates.
Main Methods:
- Population-based study utilizing multiple, overlapping search strategies and detailed record reviews.
- Identification of all pediatric cases of PSC, ASC, AIH, and inflammatory bowel disease (IBD) within a defined region.
- Analysis of demographic data, diagnostic age, incidence, prevalence, follow-up duration, disease complications, and survival rates.
Main Results:
- Identified 607 IBD, 29 PSC, 12 ASC, and 44 AIH cases. Mean ages at diagnosis were 13.0 (PSC), 11.3 (ASC), and 9.8 (AIH) years.
- Incidence/prevalence per 100,000 children: PSC (0.2/1.5), ASC (0.1/0.6), AIH (0.4/3.0).
- Five-year probabilities of complicated liver disease: PSC (37%), ASC (25%), AIH (15%). Five-year native liver survival rates: PSC (78%), ASC (90%), AIH (87%). Cholangiocarcinoma occurred in 6.9% of PSC patients.
Conclusions:
- Immune-mediated liver diseases represent a significant source of morbidity in the pediatric population.
- This population-based study provides crucial quantitative data on the burden and natural history of pediatric IMLDs.
- Findings underscore the importance of early diagnosis and management of these conditions in children.
Unlabelled:
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.
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