Changing nomenclature for PBC: From ‘cirrhosis’ to ‘cholangitis’

@article{Beuers2015ChangingNF,
  title={Changing nomenclature for PBC: From ‘cirrhosis’ to ‘cholangitis’},
  author={Ulrich Beuers and Merrill Eric Gershwin and Robert G. Gish and Pietro Invernizzi and David E. J. Jones and Keith Lindor and Xiong Ma and Ian R. Mackay and Albert Par{\'e}s and Atsushi Tanaka and John Moore Vierling and Raoul Poupon},
  journal={Hepatology},
  year={2015},
  volume={62}
}
T he disease entity today widely called ‘primary biliary cirrhosis’ was first described by Addison & Gull in 1851 and Hanot in 1876. One hundred years after its first description, MacMahon & Thannhauser proposed the term ‘xanthomatous biliary cirrhosis’ for this disease based on the typical xanthoma formation with accumulation of cholesterol esters in the skin around the eyes in association with inflammatory destruction of small intrahepatic bile ductules leading to a biliary type cirrhosis… 
51 Citations
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Primary Biliary Cholangitis.
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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.
Primary Biliary Cholangitis
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More is needed to know about the etiology of PBC to identify more appropriate and specific biomarkers that predict the clinical course and also to know which therapies are applicable at different stages, since treatment for PBC should be individualized based on stratification of the risk for progression.
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A comprehensive review of the most recent findings on the pathogenesis, clinical manifestations and therapeutic options for Sjögren’s syndrome and primary biliary cholangitis, while stressing the common traits between these conditions.
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The epidemiology, natural history, predictive and diagnostic features, and current and emerging treatments for Primary biliary cholangitis are reviewed to provide a better understanding of PBC in this patient population.
Changes in the Epidemiology of Primary Biliary Cholangitis.
TLDR
A change in patient management based on risk-stratification tools to identify subgroups at greatest potential benefit from second-line therapies is suggested.
Fluctuations of antimitochondrial antibodies and anti-gp210 antibody in a patient with primary biliary cholangitis and Sjögren syndrome with subsequent autoimmune hemolytic anemia
TLDR
This is the first case report showing a patient with comorbid PBC and SS, as well as the sequential development of AIHA with decreased AMA and increased anti-gp210 titers; this may have been due to immunodeficiency.
Autoimmune Overlap Syndromes
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Standard guidelines for treatment of these overlap syndromes are lacking, and is considered empiric and extrapolated from data derived from the primary autoimmune liver diseases.
Liver Biopsy in Primary Biliary Cholangitis: Indications and Interpretation.
Primary biliary cholangitis is a disease characterized by immune-mediated bile duct destruction, followed by inflammation, scarring, and the development of chronic cholestasis and a slow progression
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