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Immunological Mechanisms

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Scleroderma

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Abstract

Systemic sclerosis (SSc) is classically included within the spectrum of autoimmune systemic disorders. Two are the main historical reasons. One is the high prevalence of antinuclear autoantibodies (autoAb) in the sera of individuals suffering from SSc, which for several decades has been taken per se as a proof of autoimmunity. The second is that clinical features characteristically present in SSc are shared with other autoimmune systemic conditions such as systemic lupus erythematousus (SLE), rheumatoid arthritis (RA), or overlapping syndromes including mixed connective tissue disease and overlaps with myositis. It should be noted though that there is no established animal model able to reproduce the clinical phenotype of SSc upon immunization or passive transfer of immune cells or antibodies. Thus, SSc falls short of satisfying the postulates that allow a nosologic entity to be definitively classified within the disorders having an autoimmune origin. Nonetheless, during the last three decades an enormous amount of work has provided evidence indicating that different cells and soluble mediators belonging to the immune system present abnormalities that correlate with distinct SSc phenotypes and may be pathologically linked to disease development. It is however a challenge to reconstruct the chain of events leading to the perturbed state and attribute them a causal rather than a consequential role.

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Authors and Affiliations

  1. Department of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Francesco Boin MD

  2. Department of Immunology and Allergy, Geneva University Hospital, Geneva, Switzerland

    Carlo Chizzolini MD

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  1. Francesco Boin MD
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  2. Carlo Chizzolini MD
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Correspondence to Francesco Boin MD .

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  1. Feinberg School of Medicine, Northwestern University, E. Huron Street 240, Chicago, 60611-2909, Illinois, USA

    John Varga

  2. Royal Free and University College, Medical School, University College London, Rowland Street, London, NW3 2PF, United Kingdom

    Christopher P. Denton

  3. School of Medicine, Div. Rheumatology, Johns Hopkins University, Eastern Avenue 5200, Baltimore, 21224, Maryland, USA

    Fredrick M. Wigley

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Boin, F., Chizzolini, C. (2012). Immunological Mechanisms. In: Varga, J., Denton, C., Wigley, F. (eds) Scleroderma. Springer, Boston, MA. https://doi.org/10.1007/978-1-4419-5774-0_15

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