Treatment options for primary biliary cirrhosis and primary sclerosing cholangitis

@article{Levy2003TreatmentOF,
  title={Treatment options for primary biliary cirrhosis and primary sclerosing cholangitis},
  author={Cynthia Levy and Keith D. Lindor},
  journal={Current Treatment Options in Gastroenterology},
  year={2003},
  volume={6},
  pages={93-103}
}
  • C. Levy, K. Lindor
  • Published 1 April 2003
  • Medicine
  • Current Treatment Options in Gastroenterology
Opinion statementPrimary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are chronic cholestatic liver diseases that affect 0.5 to 40 per 100,000 and 1 to 6 per 100,000 Americans, respectively. Prompt recognition and management of the clinical manifestations of these diseases is essential for the patients’ well-being and ultimate outcome. Ursodeoxycholic acid (UDCA), 13 to 15 mg/kg per day, is the standard therapy for PBC and should be offered to every patient. It has been… 
Primary sclerosing cholangitis: updates in diagnosis and therapy.
Primary sclerosing cholangitis (PSC) is a chronic cholestatic syndrome of unknown origin mostly found in males, and characterized by diffuse inflammation and fibrosis of both intra- and extra-hepatic
Primary sclerosing cholangitis: role of extrahepatic biliary resection.
Liver Transplantation in Precirrhotic Biliary Tract Disease: Portal Hypertension is Frequently Associated With Nodular Regenerative Hyperplasia and Obliterative Portal Venopathy
TLDR
It is indicated that precirrhotic portal hypertension is a predominant or major contributing factor to OLT in a significant minority (11 of 306, 3.3%) of patients with chronic biliary tract disease.
Cholestatic syndromes
TLDR
Important new studies have been published about the pathogenesis, clinical features, diagnosis, and treatment of primary biliary cirrhosis, primary sclerosing cholangitis, cholestasis of pregnancy, total parenteral nutrition-induced cholESTasis, drug-inducedCholestatic syndromes, and viral cholesteins.
Gastroenterology services in the UK. The burden of disease, and the organisation and delivery of services for gastrointestinal and liver disorders: a review of the evidence
TLDR
The evidence needed to fill the void created by the absence of a national framework or guidance for service provision for the management of patients with gastrointestinal and hepatic disorders is drawn together, based on available evidence.
Etiopathogenesis of pruritus due to systemic causes: Implications for treatment.
TLDR
The understanding of the pathomechanism of pruritus of systemic origin has considerably improved in the last few years and drugs based on these mechanisms are likely to produce better results than the use of antihistamines alone.
An open, randomized, controlled study of transdermal hormone replacement therapy on the rate of bone loss in primary biliary cirrhosis
TLDR
The primary aim was to study the effect of transdermal HRT in combination with daily vitamin D and calcium supplementation on bone loss compared with vitamin D & calcium supplementation only in postmenopausal women with PBC.
Incidence and mortality of primary liver cancer in England and Wales: changing patterns and ethnic variations.
TLDR
Although approximately 30% of the PLC registrations had information on ethnicity, there was a relatively higher registration of the major tumour subtypes in patients whose ethnic backgrounds were from high incident regions of the world.
Trehalose reduces bone loss in experimental biliary cirrhosis rats via ERK phosphorylation regulation by enhancing autophagosome formation
  • Xingquan Xu, Rongliang Wang, D. Shi
  • Biology, Medicine
    FASEB journal : official publication of the Federation of American Societies for Experimental Biology
  • 2020
TLDR
Trehalose could decrease osteoblast‐mediated osteoclastogenesis and reduce PBC‐related bone loss by regulating ERK phosphorylation via autophagosome formation.
...
1
2
...

References

SHOWING 1-10 OF 87 REFERENCES
Endoscopic therapy in primary sclerosing cholangitis: outcome of treatment and risk of cancer.
TLDR
Endoscopic therapy in primary sclerosing cholangitis is indicated in selected patients and it is important to find techniques for identifying patients at risk in order to perform liver transplantation before malignant transformation.
Biliary malignancies in primary sclerosing cholangitis: Timing for liver transplantation
TLDR
Early timing of transplantation in patients with PSC is suggested to prevent formation of biliary malignancies, and regular scoring of patients with the Mayo Model risk score should be taken into consideration at scores above 4.4.
Liver transplantation for primary biliary cirrhosis and primary sclerosing cholangitis: does medical treatment alter timing and selection?
TLDR
Medical therapies for chronic cholestatic liver disease have very little effect on disease progression and, subsequently, on the timing or selection for transplantation.
Recurrence of primary sclerosing cholangitis after liver transplantation
  • I. Graziadei
  • Medicine
    Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society
  • 2002
TLDR
In conclusion, several studies provided convincing evidence that PSC recurs after OLT, with an incidence of 5% to 20% and an interval to diagnosis of at least 1 year after OLM, and recurrent disease did not influence patient or graft survival after a mean follow‐up of 4.5 years.
Long‐term results of patients undergoing liver transplantation for primary sclerosing cholangitis
TLDR
The data show that liver transplantation provides excellent long‐term patient and graft survival for patients with end‐stage PSC, and patients with associated inflammatory bowel disease did not adversely impact patient outcome posttransplantation.
Tacrolimus (FK 506), a treatment for primary sclerosing cholangitis: results of an open-label preliminary trial.
TLDR
Tacrolimus (FK 506), a new and powerful immunosuppressive macrolide antibiotic, has been used to treat 10 patients with primary sclerosing cholangitis and it is anticipated that FK 506 will become an important agent for the treatment of patients with PSC because of its powerful immunOSuppressive activity.
Long‐term ursodeoxycholic acid delays histological progression in primary biliary cirrhosis
TLDR
In conclusion, long‐term UDCA therapy appeared to delay the development of cirrhosis in PBC.
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.
Utilization of the Mayo risk score in patients with primary biliary cirrhosis receiving ursodeoxycholic acid.
TLDR
Suboptimal responders to UDCA can be identified by assessment of serum alkaline phosphatase levels, and/or Mayo risk score, and the Mayo survival model accurately predicts the clinical course in patients with PBC receiving UDCA.
...
1
2
3
4
5
...