Primary biliary cirrhosis

@article{Lindor2009PrimaryBC,
  title={Primary biliary cirrhosis},
  author={Keith D. Lindor and Merrill Eric Gershwin and Raoul Poupon and Marshall M. Kaplan and Nora V. Bergasa and Elizabeth Jenny Heathcote},
  journal={Hepatology},
  year={2009},
  volume={50}
}
Primary biliary cirrhosis was described in detail by Popper et al. in 1965. Segmentary necrosis of the interlobular bile ducts leads to progressive cholestasis of the mechanical type. Three stages, asymptomatic, cholestatic and finally terminal, are well known. The disease appears to be induced by genetic as well as by infectious and immunologic factors. There is a higher incidence of some HLA groups and sulphoxidation is deficient. Viruses such as HCV and CMV have been shown to be capable of… 
Primary biliary cholangitis
  • A. Parés
  • Medicine
    Medicina Clínica (English Edition)
  • 2018
TLDR
Primary cholangitis (cirrhosis) is a chronic cholestatic disease with an unquestionable female predominance and Ursodeoxycholic acid is the specific treatment with an excellent response in more than 60% of patients.
Diagnosis of primary biliary cirrhosis.
  • G. Hirschfield
  • Medicine
    Best practice & research. Clinical gastroenterology
  • 2011
TLDR
Liver biopsy remains useful in certain settings where clarity is needed to confirm diagnosis, exclude alternative disease, and assess the relative contribution of PBC to other co-existent liver injury, and seeks to demonstrate in particular the classic bile duct lesions, as well as the degree of interface activity.
Primary biliary cirrhosis
TLDR
Recently, animal models have been discovered that may provide a new insight into the pathogenesis of this disease and facilitate appreciation for novel treatment in PBC.
Primary biliary cholangitis
TLDR
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.
Autoimmune Liver Diseases: Primary Biliary Cholangitis
TLDR
Ursodeoxycholic acid (UDCA) is the only drug approved for the treatment of primary biliary cirrhosis, and patients with incomplete response to UDCA are at high risk for adverse events.
Options for Treatment of Primary Biliary Cirrhosis
TLDR
Primary biliary cirrhosis is a chronic progressive cholestatic disease where there is progressive, granulomatous destruction of the middle-sized bile ducts and the mainstays are the bile acid binding agents such as colestyramine.
Primary biliary cirrhosis
TLDR
Evidence supporting the autoimmune nature of primary biliary cirrhosis includes the appearance of highly specific antimitochondrial antibodies (AMAs) and autoreactive T cells and findings of epidemiological studies and murine models suggest a possible role for exogenous chemicals and infectious agents through molecular mimicry.
Primary biliary cirrhosis: Pathophysiology, clinical presentation and therapy.
TLDR
A variant, called PBC-autoimmune hepatitis (AIH) overlap, is characterized by a triad of chronic cholestasis, circulating anti-mitochondrial antibodies (AMA), and characteristic liver biopsy findings of nonsuppurative destructive cholangitis and interlobular bile duct destruction.
Primary Biliary Cholangitis: advances in management and treatment of the disease.
  • P. Invernizzi, A. Floreani, +12 authors D. Alvaro
  • Medicine
    Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver
  • 2017
TLDR
The present paper illustrates the opinion of a working group, composed of clinical pharmacologists, gastroenterologists/hepatologists with specific expertise on Primary Biliary Cholangitis and patient associations, on the state of the art and future perspectives of the disease management.
Primary biliary cirrhosis: Clinical and laboratory criteria for its diagnosis.
TLDR
This review describes the major clinical manifestations of the disease and the mechanisms of its development and presents the immunological, biochemical, and morphological signs of PBC and their significance for its diagnosis.
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TLDR
The increased family history of PBC, and the earlier onset of disease in second generation members, suggests that environmental agents are an important risk factor for the development of disease.
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TLDR
Better understanding of the pathogenesis of primary biliary cirrhosis is needed to develop specific immunotherapies and treatments to arrest the disease's progress have included corticosteroids, azathioprine, cyclosporin, and D-penicillamine.
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TLDR
Nodular hyperplastic changes are very common in early histological stages of PBC, and consequently, this disease should be considered in the etiology of NRH, which may contribute to early development of portal hypertension in patients with NRH.
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TLDR
The possibility that there may be an infectious aetiology of primary biliary cirrhosis is discussed, suggesting that the biliary epithelial cells are the main targets of disease and the response to immunosuppression is at best poor.
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TLDR
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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TLDR
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TLDR
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TLDR
In patients with precirrhotic primary biliary cirrhosis, immunosuppressive therapy with cyclosporine is promising and deserves further evaluation.
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