Primary biliary cirrhosis

@article{Carey2003PrimaryBC,
  title={Primary biliary cirrhosis},
  author={Elizabeth J Carey and Ahmad Hassan Ali and Keith D. Lindor},
  journal={The Lancet},
  year={2003},
  volume={386},
  pages={1565-1575}
}

Primary biliary cholangitis: a comprehensive overview

Serologically, PBC is characterized by presence of antimitochondrial antibodies, which are present in 90–95 % of patients and are often detectable years before clinical signs appear.

Diseases of the Liver: Primary Biliary Cholangitis

Patients with advanced- or end-stage liver disease (ESLD) should be considered for liver transplantation (LT), which is associated with excellent graft and patient outcomes, and the use of adjunct therapies is becoming more prevalent.

Primary biliary cholangitis

The survival rate of PBC patients ranged from 7.5 to 16 years, however, it has improved significantly with ursodeoxycholic acid and obeticholic acid treatment, and if there is no effect from treatment and end-stage liver failure sets in, liver transplantation is performed.

Orphan drugs in development for primary biliary cirrhosis: challenges and progress

Obeticholic acid is a first-in-class farnesoid X receptor agonist that has been recently evaluated in PBC patients with inadequate response to UDCA, and demonstrated beneficial results in improving liver biochemistries.

Diagnosis and management of primary biliary cirrhosis

Clinical trials have shown that the use of ursodeoxycholic acid in PBC results in reduction of liver biochemistries, a delay in histological progression, adelay in the development of varices and improvement in survival without liver transplantation.

Diagnosis and treatment of primary biliary cholangitis

Primary biliary cholangitis is a cholestatic, chronic autoimmune liver disease with a wide individual variation in disease progression, and preliminary data suggest an additive effect of triple therapy with ursodeoxycholic acid, obeticholic Acid, and bezafibrate.

Primary biliary cholangitis.

  • A. Parés
  • Medicine, Biology
    Medicina clinica
  • 2018

Cholestatic Liver Disease: Current Treatment Strategies and New Therapeutic Agents

The general natural history of PBC and PSC is presented, information on the latest drug therapies currently available and those that are under investigation are provided, and the design of clinical trials is provided.
...

References

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Primary biliary cirrhosis

The pathology of primary biliary cirrhosis and autoimmune cholangitis.

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Review of all small-duct biliary diseases suggests that interlobular and adjacent septal bile ducts (1st and 2nd generation ducts) represent an immunosensitive portion of the biliary tree.

Primary biliary cirrhosis: New thoughts on pathophysiology and treatment

A multicenter controlled trial is being established to determine whether patients with PBC derive significant benefit from this new line of investigation and management, after cloned a retroviral sequence directly from biliary epithelium extracted from PBC livers.

A controlled trial of cyclosporine in the treatment of primary biliary cirrhosis.

In patients with precirrhotic primary biliary cirrhosis, immunosuppressive therapy with cyclosporine is promising and deserves further evaluation.

Does Primary Biliary Cirrhosis in Men Differ from Primary Biliary Cirrhosis in Women?

Clinical findings and symptomatic status, in addition to biochemical and histopathological features, were generally similar in both male and female patients and the possible significance of higher serum alkaline phosphatase activities and lower frequency of occurrence of piecemeal necrosis in men with primary biliary cirrhosis, as compared with women, requires further study.

Primary biliary cirrhosis–autoimmune hepatitis overlap syndrome: Clinical features and response to therapy

In patients with PBC, overlap syndrome with AIH is not rare; flares of AIH may occur either spontaneously or under UDCA; and combination of UDCA and corticosteroids is required in most patients to obtain a complete biochemical response.
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