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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Primary sclerosing cholangitis: A review and update.
Primary Sclerosing Cholangitis, Part 1: Epidemiology, Etiopathogenesis, Clinical Features, and Treatment.
TLDR
A review and update of the epidemiology, etiopathogenesis, clinical features, and treatment of primary sclerosing cholangitis is provided.
Primary sclerosing cholangitis and the microbiota: current knowledge and perspectives on etiopathogenesis and emerging therapies
TLDR
A summary of and perspectives regarding the relevant basic, translational, and clinical data are provided, which encourage further investigation of the role of the microbiota and microbial metabolites in the etiopathogenesis of PSC and as a potential target for novel pharmacotherapies.
Therapies for Primary Sclerosing Cholangitis: Vancomycin as a Novel Treatment Option
TLDR
Information gathered from published case reports/series and randomised control trials on the relationship between the microbiota and PSC pathogenesis is presented with a purpose of understanding whether vancomycin is a potential effective pharmacotherapy for patients with this disease.
Pharmacologic management of primary sclerosing cholangitis: what’s in the pipeline?
TLDR
The rarity of this condition and poor understanding of its pathophysiology have created a void for safe and effective treatment options to alter mortality or transplant free survival, Nevertheless, some agents currently being tested have demonstrated therapeutic potential.
Endoscopic management of primary sclerosing cholangitis
TLDR
A narrative review on endoscopic management of PSC, including established and evolving applications to the diagnosis and treatment of both its benign and malignant complications is provided.
Ursodeoxycholic acid in primary sclerosing cholangitis: If withdrawal is bad, then administration is good (right?)
TLDR
It is clear that both its public health and patient-level burden are substantial; indeed, PSC represents a major risk factor for cholangiocarcinoma (CCA), carries a median liver transplantation (LT)-free survival of 15 years, and is a leading indication for LT in countries worldwide, despite its rarity.
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.
Cancer risk in primary sclerosing cholangitis: Epidemiology, prevention, and surveillance strategies
TLDR
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.
TLDR
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

SHOWING 1-10 OF 138 REFERENCES
The immunobiology of primary sclerosing cholangitis
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
  • K. Lankarani
  • Medicine
    Alimentary pharmacology & therapeutics
  • 2005
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.
New Treatment Strategies for Primary Sclerosing Cholangitis
  • K. Lindor
  • Medicine, Biology
    Digestive Diseases
  • 2011
TLDR
There are multiple potential therapeutic avenues to explore for patients with PSC, and it is hoped that one of these will lead to identification of a proven therapy for this disease.
Etiopathogenesis of primary sclerosing cholangitis.
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown etiology but lymphocytic portal tract infiltration is suggestive of an immune-mediated basis for this disease.…
A Pilot Study of Etanercept in the Treatment of Primary Sclerosing Cholangitis
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.
Challenges of Cholangiocarcinoma Detection in Patients with Primary Sclerosing Cholangitis
TLDR
A focused review of the current literature regarding CCA surveillance in patients with PSC is provided and the diagnostic and management challenges that exist are discussed.
Antibiotics for the Treatment of Primary Sclerosing Cholangitis
TLDR
Antibiotics have been used in several case series over the years to treat PSC and have had, in some cases, impressive benefits when compared with other drugs used for treating the disease.
Therapeutic benefit of sulfasalazine for patients with primary sclerosing cholangitis
TLDR
This is the first report describing the possible therapeutic benefit of SASP alone for adult PSC patients and describes patients with PSC associated with ulcerative colitis having biochemical and/or histological improvement of their liver disease following administration of sulfasalazine (SASP).
Will we ever model PSC? - "it's hard to be a PSC model!".
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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