Primary sclerosing cholangitis and the path to translation

Primary sclerosing cholangitis (PSC) is a rare liver disease characterized by inflammation and fibrosis of the bile ducts. Despite advancements in diagnosis, PSC remains a clinically challenging disease with a higher risk of complications and malignancies. Liver transplantation is the main treatment...

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Bibliographic Details
Published inThe Journal of clinical investigation Vol. 133; no. 17; pp. 1 - 3
Main Authors Vancza, Lorand, Torok, Natalie J
Format Journal Article
LanguageEnglish
Published Ann Arbor American Society for Clinical Investigation 01.09.2023
Subjects
Acids
Bile
Bile ducts
Biomarkers
Biomedical research
Cholangiocarcinoma
Cholangitis
Clinical trials
Development and progression
Diagnosis
Drug therapy
Dysbacteriosis
Enzymes
Feces
Fibrosis
Gastrointestinal diseases
Genetic aspects
Immune response
Inflammation
Inflammatory bowel disease
Liver
Liver diseases
Liver transplantation
Lymphocytes
Malignancy
Microbiota
miRNA
Mucosal immunity
Pathogenesis
Patients
Risk factors
Transcriptomics
Viewpoint
United States
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Summary:Primary sclerosing cholangitis (PSC) is a rare liver disease characterized by inflammation and fibrosis of the bile ducts. Despite advancements in diagnosis, PSC remains a clinically challenging disease with a higher risk of complications and malignancies. Liver transplantation is the main treatment option, but disease recurrence is a concern. The exact cause of PSC is unknown, but it likely involves gut dysbiosis, altered bile acid composition, and unidentified toxins triggering dysregulated immune responses in the liver. Recent studies have shed light on the role of microbiota, mucosal immunity, and biomarkers in PSC. High-throughput methods and spatial transcriptomics can aid in biomarker discovery and identifying new immune cell subsets. It is crucial to identify patients at high risk for disease progression or cholangiocarcinoma development. Improved surveillance using serum microRNA profiles or AI-based technologies can help predict disease outcomes. With ongoing research, effective medical therapies targeting immune dysregulation, fibrosis, and dysbiosis are expected to become available in the near future. Combination therapies and personalized approaches based on genetic susceptibilities may also be explored.
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ISSN:1558-8238
0021-9738
1558-8238
DOI:10.1172/JCI174218