Phenotypes of Primary Sclerosing Cholangitis and Differential Diagnosis.
Autoimmune overlap syndrome
IgG4-related disease
Primary sclerosing cholangitis
Secondary sclerosing cholangitis
Journal
Clinics in liver disease
ISSN: 1557-8224
Titre abrégé: Clin Liver Dis
Pays: United States
ID NLM: 9710002
Informations de publication
Date de publication:
02 2024
02 2024
Historique:
medline:
13
11
2023
pubmed:
10
11
2023
entrez:
9
11
2023
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 37945155
pii: S1089-3261(23)00086-7
doi: 10.1016/j.cld.2023.07.006
pii:
doi:
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
143-155Informations de copyright
Copyright © 2023 Elsevier Inc. All rights reserved.