Primary sclerosing cholangitis: a review and update on therapeutic developments

@article{Tabibian2013PrimarySC,
  title={Primary sclerosing cholangitis: a review and update on therapeutic developments},
  author={James H. Tabibian and Keith D. Lindor},
  journal={Expert Review of Gastroenterology \& Hepatology},
  year={2013},
  volume={7},
  pages={103 - 114}
}
  • J. Tabibian, K. Lindor
  • Published 1 February 2013
  • Medicine, Biology
  • Expert Review of Gastroenterology & Hepatology
Primary sclerosing cholangitis (PSC) is a chronic, cholestatic, idiopathic liver disease characterized by fibro-obliterative inflammation of the hepatic bile ducts. In a clinically significant proportion of patients, PSC progresses to cirrhosis, end-stage liver disease, and in some cases, cholangiocarcinoma. Despite clinical trials of nearly 20 different pharmacotherapies over several decades, safe and effective medical therapy, albeit critically needed, remains to be established. PSC is… 
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TLDR
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TLDR
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TLDR
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TLDR
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Cholangiocarcinoma in patients with primary sclerosing cholangitis.
TLDR
Despite ongoing advancements in the study of PSC-associated CCA, early diagnosis of CCA remains difficult, treatment options are limited, and prognosis is often consequently poor; Continued research in the development of high-accuracy diagnostic tools, novel biomarkers, and surveillance techniques may help to increase the likelihood of diagnosing CCA at earlier stages.
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The epidemiology, prevention, and surveillance of cancers in patients with primary sclerosing cholangitis are discussed and pragmatic approaches based on currently available data and expert opinion are presented.
Primary Sclerosing Cholangitis, Part 2: Cancer Risk, Prevention, and Surveillance.
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Cancer risk, prevention, and surveillance in patients with primary sclerosing cholangitis are reviewed and pragmatic recommendations based on current data and expert opinion are made.
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References

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TLDR
There is no effective therapy for PSC and developing a rational therapeutic strategy demands a better understanding of the disease, which may be due to dysregulation of immunity rather than a classic autoimmune disease.
Mycophenolate mofetil for the treatment of primary sclerosing cholangitis
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  • Medicine
    Alimentary pharmacology & therapeutics
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TLDR
As PSC seems to be a progressive disease passing through several stages before development of irreversible fibrosis, it is reasonable to assume that any immunosuppressive treatment could be effective only if started early in the course of disease before irreversible stricturing develops.
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  • Medicine, Biology
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TLDR
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TLDR
Etanercept, at the dosage used, was well tolerated but not effective in the treatment of PSC, although it may be helpful in treating pruritus due to cholestasis.
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TLDR
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TLDR
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TLDR
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Long-term Treatment of Primary Sclerosing Cholangitis in Children With Oral Vancomycin: An Immunomodulating Antibiotic
TLDR
Oral vancomycin could be an effective long-term treatment of sclerosing cholangitis in children, especially those without cirrhosis, and all 14 patients showed improvement in their alanine aminotransferase, γ-glutamyl transpeptidase, erythrocyte sedimentation rate, and clinical symptoms with oral vancomYcin treatment.
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