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Complement System and Systemic Lupus Erythematosus

  • Chapter
Lupus

Part of the book series: Contemporary Immunology ((CONTIM))

Abstract

Approximately 90% of individuals with complete deficiency of Clq or Clr/C1s or C4 develop SLE (1–4). Thus, total deficiency of an early component of the classical pathway of complement activation is sufficient by itself to cause SLE. Moreover, one-third of patients with C2 deficiency (C2D) develop SLE. The clinical syndrome associated with C2D is usually milder than that observed with Clq or C4 deficiency (1–3),and the biochemical defect is as well in that C2D can be readily bypassed in the complement cascade (5,6). These experiments of nature are observed on multiple HLA backgrounds and across ethnic and racial groups. It is this remarkable association that underlies the subsequent discussion.

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Authors
  1. John P. Atkinson
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  2. Jürg A. Schifferli
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Editor information

Editors and Affiliations

  1. Section on Rheumatology, Department of Internal Medicine, The Wake Forest University School of Medicine, Winston-Salem, NC, USA

    Gary M. Kammer MD

  2. Division of Rheumatology and Immunology, Uniformed Services University of Health Sciences, Bethesda, MD, USA

    George C. Tsokos MD

  3. Department of Clinical Physiology, Walter Reed Army Institute of Research, Washington, DC, USA

    George C. Tsokos MD

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Atkinson, J.P., Schifferli, J.A. (1999). Complement System and Systemic Lupus Erythematosus. In: Kammer, G.M., Tsokos, G.C. (eds) Lupus. Contemporary Immunology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-59259-703-1_32

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  • DOI: https://doi.org/10.1007/978-1-59259-703-1_32

  • Publisher Name: Humana Press, Totowa, NJ

  • Print ISBN: 978-1-4757-5686-9

  • Online ISBN: 978-1-59259-703-1

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