Autoimmune Sclerosing Cholangitis: a Review of the Overlap between Autoimmune Hepatitis and Primary Sclerosing Cholangitis in Children
Purpose of Review
Pediatric patients with autoimmune liver disease (ALD) frequently demonstrate clinical, histopathologic, cholangiographic, and serologic overlap between primary sclerosing cholangitis and autoimmune hepatitis, termed autoimmune sclerosing cholangitis (ASC). Several large, collaborative studies have shed light on the epidemiology and natural history of ASC in the last 5 years. A detailed genetic and environmental description of the pathogenesis remains lacking however. Here, we review recent advancements in knowledge on ASC in children, and their implications on the management of this rare disease.
Consensus diagnostic criteria for ASC do not exist, and we recommend labeling a patient as ASC only when clinical criteria for each of primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) are separately met. Treatment of ASC is based on the lobular component of inflammation, which behaves similar to AIH and responds to standard immunosuppression regimens. The cholangiopathy of ASC behaves like PSC and currently dictates prognosis as there is no effective therapy and no response to immunosuppression. Rates of liver transplantation for ASC are identical to those of PSC.
Rather than specifically labeling patients as ASC, the most important tasks for clinicians managing ALD are to detect treatable autoimmune lobular inflammation in PSC patients and to identify cholangiopathy in AIH patients with persistent biochemical abnormalities prior to therapeutic escalation.
KeywordsPSC ASC Primary autoimmune sclerosing cholangitis
Compliance with Ethical Standards
Conflict of Interest
C. Jaramillo, P. L. Valentino, and M. Deneau each declare no potential conflicts of interest.
Human and Animal Rights and Informed Consent
This article contains no studies with human or animal subjects performed by any of the authors.
- 2.Valentino PL, Wiggins S, Harney S, Raza R, Lee CK, Jonas MM. The natural history of primary sclerosing cholangitis in children: a large single center longitudinal cohort study. J Pediatr Gastroenterol Nutr. 2016;Google Scholar
- 4.Sheiko MA, Sundaram SS, Capocelli KE, Pan Z, McCoy AM, Mack CL. Outcomes in pediatric autoimmune hepatitis and significance of azathioprine metabolites. J Pediatr Gastroenterol Nutr. 2017.Google Scholar
- 7.Deneau MR, El-Matary W, Valentino PL, Abdou R, Alqoaer K, Amin M, et al. The natural history of primary sclerosing cholangitis in 781 children: a multicenter, international collaboration. Hepatology 2017.Google Scholar
- 14.Sebode M, Hartl J, Vergani D, Lohse AW. International Autoimmune Hepatitis Group. Autoimmune hepatitis: from current knowledge and clinical practice to future research agenda. Liver Int. 2017.Google Scholar
- 18.Shiau H, Ihekweazu FD, Amin M, Fofanova T, Miloh T, Kellermayer R. The unique inflammatory bowel disease phenotype of pediatric primary sclerosing cholangitis: a single center study. J Pediatr Gastroenterol Nutr. 2017.Google Scholar
- 24.Iwasawa K, Suda W, Tsunoda T, Oikawa-Kawamoto M, Umetsu S, Inui A, et al. Characterisation of the faecal microbiota in Japanese patients with paediatric-onset primary sclerosing cholangitis. Gut 2016.Google Scholar
- 25.Smolka V, Karaskova E, Tkachyk O, Aiglova K, Ehrmann J, Michalkova K, et al. Long-term follow-up of children and adolescents with primary sclerosing cholangitis and autoimmune sclerosing cholangitis. Hepatobiliary & Pancreatic Diseases International: HBPD INT. 2016;15(4):412–8.CrossRefGoogle Scholar
- 27.Zizzo AN, Valentino PL, Shah PS, Kamath BM. Second-line agents in pediatric patients with autoimmune hepatitis: a systematic review and meta-analysis. J Pediatr Gastroenterol Nutr. 2017.Google Scholar