Ursodeoxycholic acid in primary sclerosing cholangitis: If withdrawal is bad, then administration is good (right?)

@article{Tabibian2014UrsodeoxycholicAI,
  title={Ursodeoxycholic acid in primary sclerosing cholangitis: If withdrawal is bad, then administration is good (right?)},
  author={James H. Tabibian and Keith D. Lindor},
  journal={Hepatology},
  year={2014},
  volume={60}
}
P rimary sclerosing cholangitis (PSC) is a chronic, idiopathic, cholestatic liver disease characterized by biliary inflammation and periductal fibrosis. Unlike most other cholestatic liver diseases, PSC affects individuals of essentially all ages and racial backgrounds, remains etiopathogenically perplexing, and lacks established medical therapy despite extensive laboratorybased investigation, translational research, and clinical trials. It is on this basis that PSC has, regrettably, come to be… 
Primary Sclerosing Cholangitis
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.
New Therapeutic Strategies for Primary Sclerosing Cholangitis.
TLDR
It is likely that an effective drug therapy for PSC will become available over the next decade, and several agents are in development, targeting various receptors, integrins, and ligands on this pathway, including VAP-1, MAdCAM- 1, α4β7, and CCR9.
Primary sclerosing cholangitis: diagnostic and management challenges
TLDR
The diagnostic and management challenges of PSC are addressed, with a succinct analysis of existing therapies, their limitations, and a glimpse into the future of the management of this multifaceted pathologic entity.
Therapy of Primary Sclerosing Cholangitis - Today and Tomorrow
TLDR
The current status and recent progress in the development of targeted therapeutic approaches based on increasing knowledge about the pathogenesis of primary sclerosing cholangitis are summarized.
An Overview on Primary Sclerosing Cholangitis
TLDR
Continuous screening consists of annual clinical, biochemical, and ultrasound assessments in asymptomatic patients and annual colonoscopy in patients with PSC and inflammatory bowel disease and further studies are in progress to establish the effect of molecular-targeted therapies in PSC.
Current Concepts in Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis
TLDR
Primary biliary cirrhosis and primary sclerosing cholangitis are chronic, cholestatic diseases of the liver with common clinical manifestations and close surveillance is indicated.
Treatment of primary sclerosing cholangitis in children
TLDR
The evidence for drug efficacy, dosing, duration of therapy, and treatment targets in PSC is reviewed, and a framework for endoscopic and medical management of this complex problem is provided.
Emerging treatments for primary sclerosing cholangitis
TLDR
This review will explore previously tested treatments, discuss current treatment strategies and present viewpoints about future emerging therapies in PSC, an uncommon and highly heterogeneous disease associated with inflammatory bowel disease and a complex pathophysiology.
...
...

References

SHOWING 1-10 OF 24 REFERENCES
Primary sclerosing cholangitis: a review and update on therapeutic developments
TLDR
The authors herein provide a review of previously tested pharmacologic agents, discuss emerging fundamental concepts and present viewpoints regarding how identifying therapies for PSC may evolve over the next several years.
High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis
TLDR
The majority of trials using UDCA in PSC are underpowered and fail to show improvements in clinically relevant endpoints, such as delayed progression to cirrhosis, portal hypertension, liver transplantation, development of cholangiocarcinoma, or death.
A preliminary trial of high-dose ursodeoxycholic acid in primary sclerosing cholangitis.
TLDR
High-dose UDCA may be of clinical benefit in PSC, but trials with a larger number of participants and of longer duration are required to establish whether the effect of high- dose UDCA on liver biochemistry, histology, and cholangiography in patients with PSC is translated into improved long-term survival.
Prospective evaluation of ursodeoxycholic acid withdrawal in patients with primary sclerosing cholangitis
TLDR
At 3 months, discontinuation of UDCA in patients with PSC causes significant deterioration in liver biochemistry and influences concentrations of bile acid metabolites, but other short‐term markers of quality of life are unaffected.
High-dose ursodeoxycholic acid in primary sclerosing cholangitis: a 5-year multicenter, randomized, controlled study.
TLDR
This study found no statistically significant beneficial effect of a higher dose of ursodeoxycholic acid than previously used on survival, symptoms, biochemistry, and quality of life in primary sclerosing cholangitis.
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.
Liver transplantation for primary sclerosing cholangitis.
A re‐evaluation of the risk factors for the recurrence of primary sclerosing cholangitis in liver allografts
  • E. Alabraba, P. Nightingale, J. Neuberger
  • Medicine
    Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society
  • 2009
TLDR
It is shown that colectomy remains a significant risk factor for rPSC and that coLECTomy before and during initial LT for PSC confers a protective effect against r PSC in subsequent graft(s).
Liver transplantation for primary sclerosing cholangitis.
TLDR
Patient and graft survivals following liver transplantation are good, with 1 year patient survival exceeding 90%.
Ursodeoxycholic acid for treatment of primary sclerosing cholangitis: A placebo‐controlled trial
TLDR
It is concluded that ursodeoxycholic acid is beneficial in reducing disease activity in patients with primary sclerosing cholangitis and histopathological features improved significantly, as evaluated by multiparametric score.
...
...