Correlation of initial autoantibody profile and clinical outcome in primary biliary cirrhosis

Authors

  • Józefa Wesierska-Gadek,

    1. First Department of Internal Medicine, Institute of Cancer Research, Vienna Medical University, Vienna, Austria
    Search for more papers by this author
  • Edward Penner,

    1. Fourth Department of Internal Medicine, Vienna Medical University, Vienna, Austria
    Search for more papers by this author
  • Pier Maria Battezzati,

    1. Division of Internal Medicine, Department of Medicine, Surgery, and Dentistry, San Paolo School of Medicine, University of Milan, Milan, Italy
    Search for more papers by this author
  • Carlo Selmi,

    1. Division of Internal Medicine, Department of Medicine, Surgery, and Dentistry, San Paolo School of Medicine, University of Milan, Milan, Italy
    2. Division of Rheumatology, Allergy, and Clinical Immunology, University of California–Davis, Davis, CA
    Search for more papers by this author
  • Massimo Zuin,

    1. Division of Internal Medicine, Department of Medicine, Surgery, and Dentistry, San Paolo School of Medicine, University of Milan, Milan, Italy
    Search for more papers by this author
  • Eva Hitchman,

    1. First Department of Internal Medicine, Institute of Cancer Research, Vienna Medical University, Vienna, Austria
    Search for more papers by this author
  • Howard J. Worman,

    1. Departments of Medicine and Anatomy and Cell Biology, College of Physicians and Surgeons, Columbia University, New York, NY
    Search for more papers by this author
  • M. Eric Gershwin,

    1. Division of Rheumatology, Allergy, and Clinical Immunology, University of California–Davis, Davis, CA
    Search for more papers by this author
  • Mauro Podda,

    1. Division of Internal Medicine, Department of Medicine, Surgery, and Dentistry, San Paolo School of Medicine, University of Milan, Milan, Italy
    Search for more papers by this author
  • Pietro Invernizzi

    Corresponding author
    1. Division of Internal Medicine, Department of Medicine, Surgery, and Dentistry, San Paolo School of Medicine, University of Milan, Milan, Italy
    • Division of Internal Medicine, Department of Medicine, Surgery, Dentistry, San Paolo School of Medicine, University of Milan, Via Di Rudinfi 8, 20142 Milan, Italy
    Search for more papers by this author
    • fax: (39)-02-50323089


  • Presented in part at the annual meeting of the European Association for the Study of the Liver in Berlin, Germany, in April 2004, and published in abstract form in J Hepatol 2004;40:159–160.

  • Potential conflict of interest: Nothing to report.

Abstract

Although there have been significant advances in understanding the clinical and biochemical features of primary biliary cirrhosis (PBC), there is still a paucity of data on the usefulness of biomarkers as prognostic indicators. This is particularly important at the time of initial diagnosis. Indeed, the widespread use of antimitochondrial antibody testing has led to an earlier diagnosis of asymptomatic PBC and it is difficult to predict which patients will experience a benign versus a rapidly progressive course. To address this issue, we examined a unique population of 127 newly diagnosed patients with PBC during a 15-year period of observation that began in January 1990. Sera from these patients were analyzed for antimitochondrial, antinuclear, and anti–smooth muscle antibodies, and immunoblotting was performed for nuclear pore complex (NPC). The patients were then followed up longitudinally using biochemical liver function tests. No patient was under any medical therapy for PBC at the time of the initial sera collection. Data were analyzed based not only on the clinical features, but also the Mayo score and specific outcome measures, including time to death, need for liver transplantation, and complication free survival. Among patients with early disease, bilirubin increased to >2 mg/dL in the anti-NPC(+) patients (26% vs. 5%, P = .019). Anti-NPC antibodies remained stable or slightly increased over the period of observation. In conclusion, anti-NPC identifies patients likely to experience an unfavorable clinical course and more rapid disease progression. (HEPATOLOGY 2006;43:1135–1144.)

Ancillary