Phenotypes of Primary Sclerosing Cholangitis and Differential Diagnosis.

Brian H Horwich, Douglas T Dieterich

Journal: Clinics in liver disease 2023;28(1):143-155

PMID: 37945155

Full Text on Elsevier Science View on PubMed

Abstract

Primary sclerosing cholangitis is a heterogenous immune-mediated disorder characterized by chronic inflammation and stricturing of the bile ducts. Though the driving pathophysiologic mechanisms remain elusive, there are several observed clinical phenotypes of the disease. The distribution of bile duct involvement, presence of concomitant inflammatory bowel disease, significant infiltration of IgG4-positive plasma cells, and overlapping features with other autoimmune disease has significant implications for prognosis and treatment. As there remains no pathognomonic finding for primary sclerosing cholangitis, a broad differential diagnosis and extensive evaluation of other underlying causes is critical to appropriate management.

Address: Division of Gastroenterology, Icahn School of Medicine at Mount Sinai, 1 Gustave L. Levy Place, PO Box 1076, New York, NY 10029, USA.; Division of Liver Diseases, Institute for Liver Medicine, Icahn School of Medicine at Mount Sinai, 1468 Madison Avenue, Annenberg 5-04, New York, NY 10029, USA. Electronic address: [email protected].

DOI: 10.1016/j.cld.2023.07.006

Link outs

Free resources

Medical:

https://medlineplus.gov/autoimmunediseases.html

Research Materials:

https://antibodies.cancer.gov/detail/CPTC-HLA-B-1

Subscription / membership required

Full Text Sources:

MeSH Terms: Humans, Cholangitis, Sclerosing, Diagnosis, Differential, Bile Ducts, Autoimmune Diseases, Phenotype