Primary biliary cirrhosis

@article{Selmi2011PrimaryBC,
  title={Primary biliary cirrhosis},
  author={Carlo Selmi and Christopher L. Bowlus and Merrill Eric Gershwin and Ross L. Coppel},
  journal={The Lancet},
  year={2011},
  volume={377},
  pages={1600-1609}
}
Primary biliary cirrhosis is a chronic liver disease characterised by intrahepatic bile-duct destruction, cholestasis, and, in some cases, cirrhosis. Evidence supporting the autoimmune nature of this disorder includes the appearance of highly specific antimitochondrial antibodies (AMAs) and autoreactive T cells. Concordance rates in monozygotic twins, familial prevalence, and genetic associations underscore the importance of genetic factors, whereas findings of epidemiological studies and… 
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.
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.
Chapter 62 – Primary Biliary Cirrhosis
TLDR
Several informative transgene-based mouse models have been developed and confirm the nexus between AMA reactivity and cholangiocyte destruction, and experimental models are available supporting the participation in pathogenesis of environmental xenobiotics.
Primary Biliary Cholangitis.
TLDR
The classic presentation of an asymptomatic patient with a cholestatic pattern of liver injury is described, including the initial work-up and differential diagnosis for this pattern, the diagnostic criteria for PBC, and specific treatment, with an emphasis on early referral to a liver specialist.
The Diagnosis and Classification of Immune-Mediated Biliary Diseases
TLDR
This chapter aims to introduce the reader to relevant themes in the diagnosis and classification of immune-mediated biliary diseases.
Primary Biliary Cholangitis: Its Science and Practice
TLDR
No “perfect model” has been developed to date for PBC pathogenesis, but novel therapeutic avenues targeting bile acids, nuclear receptors, immune cell receptors, and cytokines have been developed with promising results.
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.
Cirrhosis and autoimmune liver disease: Current understanding
TLDR
LT is indicated for AILD patients who have progressed to end-stage chronic liver disease or developed intractable symptoms or hepatic malignancy; in addition, LT may also be indicated for patients presenting with acute liver disease due to AIH who do not respond to steroids.
Cirrhosis and Autoimmune Liver Disease
TLDR
Standard monitoring for cirrhosis-related complications is highly recommended and OCA should be considered in non-responders but used with very close monitoring in patients with decompensated Cirrhosis.
Role for mycobacterial infection in pathogenesis of primary biliary cirrhosis?
TLDR
The view that mycobacteria are infectious triggers of PBC is intriguing, but the data provided so far are not conclusive.
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References

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Primary biliary cirrhosis
TLDR
The most specific feature differentiating primary biliary cirrhosis from other causes of intrahepatic cholestasis is demonstration of antimitochondrial antibodies and the typical histology.
Management of primary biliary cirrhosis
TLDR
The hydrophilic bile acid, ursodeoxycholic acid (UDCA), has been shown when given in a dose of 13 to 15 mg/kg daily for up to 4 years to delay the time to liver transplantation or death and causes a significant improvement of all the biochemical markers of cholestasis but has no beneficial effects on any of the symptoms or associated disorders.
Pathogenesis of primary biliary cirrhosis
  • D. Jones
  • Medicine
    Postgraduate Medical Journal
  • 2008
TLDR
Broadening of the diagnostic base has led us to appreciate that there is a significant subgroup within the PBC population who have a low risk of disease progression and who are unlikely to develop end-stage liver disease during a normal lifetime (but who remain at risk of developing the often life-altering symptoms of the disease).
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TLDR
There is insufficient data to support or reject the use of glucocorticosteroids for patients with primary biliary cirrhosis, and a large prospective randomised clinical trial on this topic is appropriate.
Pediatric-onset primary biliary cirrhosis.
TLDR
2 liver biopsy-confirmed cases of pediatric-onset AMA-positive primary biliary cirrhosis diagnosed at 16 and 15 years of age are presented, with increased awareness of early-onet PBC, and further pediatric cases may be discovered.
Primary biliary cirrhosis–autoimmune hepatitis overlap syndrome: Clinical features and response to therapy
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.
The X and why of xenobiotics in primary biliary cirrhosis.
TLDR
This review will discuss the current knowledge of the immunoreactivity of xenobiotically modified PDC peptide antigens and provide a working hypothesis how xenobiotic modification of antigen might occur that ultimately leads to the breaking of self-tolerance and the induction of primary biliary cirrhosis.
Does a betaretrovirus infection trigger primary biliary cirrhosis?
  • Lizhe Xu, Zhiwei Shen, +8 authors A. Mason
  • Biology, Medicine
    Proceedings of the National Academy of Sciences of the United States of America
  • 2003
TLDR
In vivo and in vitro, it is found that lymph node homogenates from patients with primary biliary cirrhosis can induce autoantigen expression in normal biliary epithelial cells in coculture, and the human betaretrovirus is referred to as the putative agent because it shares close homology with the murine mammary tumor virus and a human retrovirus cloned from breast cancer tissue.
The prognostic importance of clinical and histologic features in asymptomatic and symptomatic primary biliary cirrhosis.
TLDR
A multivariate analysis of clinical features revealed that at the onset of disease, age, hepatomegaly, and elevated levels of serum bilirubin were independent discriminators of a poor prognosis, and a histologic finding of fibrosis limited to portal areas improved this discrimination, correlating with prolonged survival.
Incidence, prevalence and clinical course of primary biliary cirrhosis in a Spanish community
TLDR
Incidence and prevalence of primary biliary cirrhosis in the reference area are higher than in some Spanish areas, as per the results previously published; however, they are comparable with those obtained in Northern Europe and the US.
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