Primary sclerosing cholangitis is rare among Chinese. We report on a 71 year old male patient who presented with clinical features consistent with the disorder. Subsequent investigations confirmed the diagnosis. The patient was found to have anti-neutrophil cytoplasmic antibodies with specificity against proteinase 3. Treatment with ursodeoxycholic acid resulted in clinical remission and disappearance of the antibodies.
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http://dx.doi.org/10.1111/j.1440-1746.1996.tb01845.x | DOI Listing |
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 PDFCurr 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.
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.
Results: Whole blood DNA methylation profiling and genotyping was performed in 264 individuals; 47 with IgG4-SC, 65 with PSC, 64 with ulcerative colitis (UC), and 88 healthy controls.
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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