Recent Insights into Pediatric Primary Sclerosing Cholangitis

James P Stevens; Nitika A Gupta

doi:10.1016/j.cld.2022.03.009

Recent Insights into Pediatric Primary Sclerosing Cholangitis

Clin Liver Dis. 2022 Aug;26(3):489-519. doi: 10.1016/j.cld.2022.03.009. Epub 2022 Jun 25.

Authors

James P Stevens¹, Nitika A Gupta²

Affiliations

¹ Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, 1760 Haygood Drive, Atlanta GA 30322, USA.
² Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, 1760 Haygood Drive, Atlanta GA 30322, USA. Electronic address: [email protected].

PMID: 35868687
DOI: 10.1016/j.cld.2022.03.009

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.

Keywords: Autoimmune sclerosing cholangitis; Immune-mediated liver disease; Pediatric liver disease; Pediatric sclerosing cholangitis; Primary sclerosing cholangitis.

Publication types

Review

MeSH terms

Cholangitis, Sclerosing* / diagnosis
Cholangitis, Sclerosing* / epidemiology
Cholangitis, Sclerosing* / etiology
Hepatitis, Autoimmune* / diagnosis
Hepatitis, Autoimmune* / epidemiology
Hepatitis, Autoimmune* / therapy
Humans
Inflammatory Bowel Diseases* / diagnosis
Inflammatory Bowel Diseases* / epidemiology
Inflammatory Bowel Diseases* / etiology
Prognosis
Prospective Studies