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Serology of Primary Biliary Cirrhosis

  • Chapter
Immunological Aspects of Liver Disease

Abstract

The aetiology of primary biliary cirrhosis (PBC) still remains unknown. Many features point towards an autoimmune disorder [25]: the female preponderance, the familial aggregation, the association with other autoimmune conditions, the protracted course, the presence of various types of antibodies (smooth muscle (SMA), nuclear (ANA), and especially mitochondrial antibodies (AMA)), and the histological lesions (showing granuloma formation and infiltration of bile ducts by lymphocytes). In addition in a high percentage of patients circulating cryoglobulins have been detected [91] and there is a striking abnormality of the complement system [45] and impairment of cell mediated immunity [62]. Also cellular immunity against biliary antigens [64] and mitochondria [18] has been described, and patients’ lymphocytes also seem to interfere in vitro with some mitochondrial functions [16, 24]. AMA apparently play no role in the pathogenesis of PBC but their incidence in almost 90% of all PBC patients led to the speculation that these antibodies could be related to the aetiology of the disease.

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Authors and Affiliations

  1. Medizinische Universitätsklinik, Otfried-Müller-Straße, D-7400, Tübingen 1, Federal Republic of Germany

    Peter A. Berg

  2. Department of Biochemistry, Chelsea College, Manresa Road, London, SW3 6LX, UK

    Harold Baum

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  1. Peter A. Berg
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Editors and Affiliations

  1. Academic Department of Medicine, The Royal Free Hospital and Medical School, Pond Street, Hampstead, London, NW3 2QG, Great Britain

    Howard C. Thomas

  2. Division d’Hématologie du Department de Médicine Hôpital Cantonal, 25, rue Micheli-du Crest, CH-1211, Genève, Switzerland

    Peter A. Miescher

  3. Department of Molecular Immunology, Scripps Clinic and Research Foundation, La Jolla, CA, 92037, USA

    Hans J. Mueller-Eberhard

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Berg, P.A., Baum, H. (1982). Serology of Primary Biliary Cirrhosis. In: Thomas, H.C., Miescher, P.A., Mueller-Eberhard, H.J. (eds) Immunological Aspects of Liver Disease. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-68446-3_7

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