Recent Insights into Pediatric Primary Sclerosing Cholangitis.

James P Stevens, Nitika A Gupta

Journal: Clinics in liver disease 2022;26(3):489-519

PMID: 35868687

Full Text on Elsevier Science View on PubMed

Abstract

This article reviews recent literature on the pathogenesis, presentation, diagnosis, comorbidities, natural history, and management of pediatric primary sclerosing cholangitis (PSC). The authors shed light on the role of genetic and environmental factors in PSC, although recognize the limitations in the understanding of PSC pathogenesis. They reflect on presenting disease phenotypes, including the association with inflammatory bowel disease and frequent histologic presence of autoimmune hepatitis features. The current lack of effective medications is discussed, and disease complications and prognosis are described. Finally, the authors highlight available evidence while acknowledging the paucity of prospective pediatric data.

Address: Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, 1760 Haygood Drive, Atlanta GA 30322, USA.; Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, 1760 Haygood Drive, Atlanta GA 30322, USA. Electronic address: [email protected].

DOI: 10.1016/j.cld.2022.03.009

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MeSH Terms: Cholangitis, Sclerosing, Hepatitis, Autoimmune, Humans, Inflammatory Bowel Diseases, Prognosis, Prospective Studies