<?xml version="1.0" encoding="UTF-8"?>
<term>
  <id>13600</id>
  <title>primary biliary cirrhosis</title>
  <longtitle>IUPAC Gold Book - primary biliary cirrhosis</longtitle>
  <doi>10.1351/goldbook.13600</doi>
  <code>13600</code>
  <status>current</status>
  <initialism><em>initialism</em>: PBC</initialism>
  <definitions>
    <item>
      <id>1</id>
      <text>Autoimmune liver disease that results in the destruction of bile ducts, leading to fibrosis and cirrhosis.</text>
      <notes>
        <item>Primary biliary cirrhosis-specific antibodies are antimitochondrial antibodies (AMAs) directed against proteins of the pyruvate dehydrogenase complex (mainly the E2 subunit).</item>
      </notes>
      <links>
        <item>
          <term>Autoimmune</term>
          <url>https://goldbook.iupac.org/terms/view/12918</url>
        </item>
        <item>
          <term>antimitochondrial antibodies (AMAs)</term>
          <url>https://goldbook.iupac.org/terms/view/12895</url>
        </item>
      </links>
      <sources>
        <item>PAC, 2012, 84, 1113. 'IUPAC glossary of terms used in immunotoxicology (IUPAC Recommendations 2012)' on page 1233 (https://doi.org/10.1351/PAC-REC-11-06-03)</item>
      </sources>
    </item>
  </definitions>
  <altoutputs>
    <html>https://goldbook.iupac.org/terms/view/13600/html</html>
    <json>https://goldbook.iupac.org/terms/view/13600/json</json>
    <plain>https://goldbook.iupac.org/terms/view/13600/plain</plain>
  </altoutputs>
  <citation>Citation: 'primary biliary cirrhosis' in IUPAC Compendium of Chemical Terminology, 5th ed. International Union of Pure and Applied Chemistry; 2025. Online version 5.0.0, 2025. 10.1351/goldbook.13600</citation>
  <license>The IUPAC Gold Book is licensed under Creative Commons Attribution-ShareAlike CC BY-SA 4.0 International (https://creativecommons.org/licenses/by-sa/4.0/) for individual terms.</license>
  <collection>If you are interested in licensing the Gold Book for commercial use, please contact the IUPAC Executive Director at executivedirector@iupac.org .</collection>
  <disclaimer>The International Union of Pure and Applied Chemistry (IUPAC) is continuously reviewing and, where needed, updating terms in the Compendium of Chemical Terminology (the IUPAC Gold Book). Users of these terms are encouraged to include the version of a term with its use and to check regularly for updates to term definitions that you are using.</disclaimer>
  <accessed>2026-06-30T07:35:48+00:00</accessed>
</term>
