Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown etiology;however, lymphocytic portal tract infiltration is suggestive of an immune-mediated basis for PSC. Associations with inflammatory bowel disease--especially ulcerative colitis--and with other auto-immune diseases, together with genetic associations, further suggest that PSC may be an immune-mediated disease. The immunogenetics of PSC have been the subject of active research, and several human leukocyte antigen (HLA)- and non-HLA-associated genes have been implicated in the development of the disease. Lymphocytes derived from the inflamed gut may enter the liver via the enterohepatic circulation to cause hepatic disease.PSC may be triggered in genetically susceptible individuals by infections or toxins entering the portal circulation through a permeable colon and, therefore, evoking an abnormal immune response.
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Ulcerative colitis can present with extra-intestinal manifestations, including interstitial lung disease and primary sclerosing cholangitis. When pulmonary symptoms precede gastrointestinal, diagnosis can be challenging. Consideration of Ulcerative colitis in patients with unexplained lung and hepatic pathology is crucial, as a failure of timely intervention can lead to multiorgan complications.
View Article and Find Full Text PDFAssessing patient-reported outcomes in primary sclerosing cholangitis: an update.
Curr Opin Gastroenterol
January 2025
Center for Health Measurement, Department of Population Health Sciences, Duke University School of Medicine, Durham, North Carolina, USA.
Purpose Of Review: Patient-reported outcome (PRO) measures validated in primary sclerosing cholangitis (PSC) are needed for clinical trials. This review describes the recent US Food & Drug Administration (FDA) Patient-Focused Drug Development (PFDD) guidelines, existing PRO measures used in PSC studies, and the design of PSC-specific symptom measures adherent with the guidelines.
Recent Findings: FDA released updated guidance reflecting best practices for the design and evaluation of clinical outcome assessments (including PROs) and the design of trial endpoints.
Epigenetic age acceleration and methylation differences in IgG4-related cholangitis and primary sclerosing cholangitis.
Clin Epigenetics
January 2025
Translational Gastroenterology and Liver Unit, John Radcliffe Hospital, Headley Way, Headington, Oxford, OX3 9DU, UK.
Background: IgG4-related cholangitis (IgG4-SC) and primary sclerosing cholangitis (PSC) are chronic fibro-inflammatory hepatobiliary conditions, with genetic, environmental, and immunologic risk factors, in which epigenetic alterations may provide insights into pathophysiology and novel biomarkers. This study is the first to assess methylation signatures in IgG4-SC.
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Impact of liver transplantation on intestinal and systemic inflammation markers in patients with colitis ulcerosa concomitant with primary sclerosing cholangitis.
Prz Gastroenterol
December 2024
Department of Infectious Diseases, Hepatology and Liver Transplantation, Pomeranian Medical University, Szczecin, Poland.
Introduction: Primary sclerosing cholangitis (PSC) is an uncommon, chronic liver disease characterised by fibrosis and strictures of a bile ducts, causing cholestasis. In the long term it can lead to complete stenosis leading in turn to liver cirrhosis. In patients with severe form of the disease, the recommended treatment is liver transplantation.
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