Primary sclerosing cholangitis, autoimmune hepatitis, and overlap in utah children: Epidemiology and natural history

Abstract

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) 5 21%-58%] for PSC, 25% (95% CI 5 7%-70%) for ASC, and 15% (95% CI 5 7%-33%) for AIH. The 5-year survival rates with the native liver were 78% (95% CI 5 54%-91%) for PSC, 90% (95% CI 5 47%-99%) for ASC, and 87% (95% CI 5 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 populationbased design, this study quantifies the burden and natural history of immune-mediated liver disease in children. (HEPATOLOGY 2013;58:1392-1400

Incidence and Characteristics of Autoimmune Hepatitis

“…Previous reports of pediatric prevalence are scarce and vary from 2.2 in non-First Nation (indigenous native Canadian) communities to 9.9 in First Nation communities per 100 000 children in British Columbia. 28 A recent report by Deneau et al 13 in Utah described an incidence of 0.4 case per 100 000 children and a prevalence of 3 per 100 000. The previously reported incidence in adults ranged from 0.1 to 1.9 per 100 000 per year (including some adolescents).…”

“…12 A recent study in pediatric patients reported an incidence of 0.4 case per 100 000 children. 13 Another study from Poland reported an incidence of 3 to 4 per 100 000 children. 14 Available adult data indicate an incidence varying from 0.67 to 2 cases per 100 000 people.…”

The management of childhood liver diseases in adulthood

“…AIH is a rare condition both in adults and children, with a reported incidence of 0.4 and 3.0/100,000 children [85,86].…”

The Association of Celiac Disease With Other Autoimmune Diseases in Children

“…Although there are still no recommendations for routine serological CD screening in patients with AIH, the association between these two diseases should be considered if one of them is diagnosed. Rare cases of CD associated with other autoimmune liver diseases such as primary sclerosing cholangitis (PSC) and "overlap" syndrome (AIH / PSC) were described in children [35,36].…”