ACG Clinical Guideline: Primary Sclerosing Cholangitis

@article{Lindor2015ACGCG,
  title={ACG Clinical Guideline: Primary Sclerosing Cholangitis},
  author={Keith D. Lindor and Kris V. Kowdley and M Edwyn Harrison},
  journal={The American Journal of Gastroenterology},
  year={2015},
  volume={110},
  pages={646-659}
}
Primary sclerosing cholangitis is a chronic cholestatic liver disease that can shorten life and may require liver transplantation. The cause is unknown, although it is commonly associated with colitis. There is no approved or proven therapy, although ursodeoxycholic acid is used by many on an empiric basis. Complications including portal hypertension, fat-soluble vitamin deficiency, metabolic bone diseases, and development of cancers of the bile duct or colon can occur. 
Primary Sclerosing Cholangitis
TLDR
Management should be treatment of symptoms and complications of cholestasis, as well as attempts at treating the underlying disease process, and efforts should be made to recognize and treat or prevent the known complications of primary sclerosing cholangitis.
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.
Primary Sclerosing Cholangitis: Therapeutic Options and Surveillance Management
TLDR
An overview of primary sclerosing cholangitis is provided with further focus on current therapeutic options and guidance on surveillance management.
Endoscopic Evaluation and Management of Primary Sclerosing Cholangitis
TLDR
The central role of endoscopy in the initial diagnosis of PSC, endoscopic evaluation and endotherapy for dominant strictures, endoscope evaluation for development of CCA, and endoscopic evaluated and management of recurrent PSC after liver transplantation are reviewed.
Primary Biliary Cholangitis and Primary Sclerosing Cholangitis.
TLDR
This review will address the general clinical features of primary biliary cholangitis and primary sclerosing cholangsitis, analyze their commonalities and differences, and provide a state-of-the art overview of the currently available therapeutics.
...
...

References

SHOWING 1-10 OF 196 REFERENCES
[Primary sclerosing cholangitis].
TLDR
During treatment with UDCA stenoses of major ducts may develop and early endoscopic dilatation is highly effective and in patients with endstage disease, UDCA is not effective and liver transplantation is indicated.
Pathogenesis and management of pruritus in cholestatic liver disease
TLDR
Novel insights are described into the pathogenesis and management of pruritus in patients with cholestasis and several mediators possibly playing an important role.
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.
Primary Biliary Cirrhosis
TLDR
The primary medical treatment in PBC should focus on reducing the rate of disease progression, and ursodeoxycholic acid has been extensively evaluated and proven to improve liver biochemistries and survival in patients with PBC.
Ursodiol for Primary Sclerosing Cholangitis
TLDR
Ursodiol (ursodeoxycholic acid) benefits patients with primary biliary cirrhosis, another cholestatic liver disease, and well-documented primary sclerosing cholangitis in a randomized, double-blind study comparing ursodiol with placebo.
Ursodiol for primary sclerosing cholangitis. Mayo Primary Sclerosing Cholangitis-Ursodeoxycholic Acid Study Group.
  • K. Lindor
  • Medicine
    The New England journal of medicine
  • 1997
TLDR
In a group of patients with well-defined primary sclerosing cholangitis, ursodiol provided no clinical benefit and was associated with improvement in serum alkaline phosphatase, aspartate aminotransferase, bilirubin, and albumin levels at one and two years.
Recurrence and rejection in liver transplantation for primary sclerosing cholangitis.
TLDR
This work has reviewed the current literature on epidemiology, pathogenesis, treatment and the possible influence of rejection on the risk of recurrent disease in the allograft affecting primary sclerosing cholangitis patients.
Tacrolimus for the treatment of primary sclerosing cholangitis
Background: Results from a pilot investigation with tacrolimus for primary sclerosing cholangitis (PSC) demonstrated biochemical improvement without excessive drug toxicity. To date, no confirmatory
...
...