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Genetics of ANCA-associated Vasculitides

  • VASCULITIS (LR ESPINOZA, SECTION EDITOR)
  • Published:
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Abstract

The distribution of the anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV) is not uniform across geographical regions and ethnic and racial groups, suggesting that genetic and environmental factors affect the pathogenesis of these diseases. In addition, genetic factors affect not only the clinical syndrome phenotypes and their prognosis, but also ANCA specificity; these data suggest that AAV may need reclassification. Several genes have been evaluated, including ANCA targets and those of the immune system, for example co-stimulatory molecules, signaling regulators, cytokines, Fc and other receptors, and other proteins. This article provides a review of genetic factors affecting the pathogenesis and prognosis of AAV. Further studies to determine the effect of genetic factors on the clinical syndrome phenotypes and ANCA specificity need to be performed across different ethnic groups.

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Acknowledgment

The authors want to acknowledge Dr Graciela S. Alarcón from Department of Medicine, Division of Clinical Immunology and Rheumatology, School of Medicine, The University of Alabama at Birmingham, Birmingham, AL, USA for her critical review of this paper.

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Conflict of Interest

Manuel F. Ugarte-Gil and Luis R. Espinoza declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

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Authors and Affiliations

  1. Servicio de Reumatología, Hospital Nacional Guillermo Almenara Irigoyen, EsSalud, Lima, Perú

    Manuel F. Ugarte-Gil

  2. Universidad Científica del Sur, Lima, Perú

    Manuel F. Ugarte-Gil

  3. Louisiana State University Health Sciences Center, 433 Bolivar St, New Orleans, LA, 70112, USA

    Luis R. Espinoza

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  1. Manuel F. Ugarte-Gil
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  2. Luis R. Espinoza
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Correspondence to Manuel F. Ugarte-Gil.

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This article is part of the Topical Collection on Vasculitis

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Ugarte-Gil, M.F., Espinoza, L.R. Genetics of ANCA-associated Vasculitides. Curr Rheumatol Rep 16, 428 (2014). https://doi.org/10.1007/s11926-014-0428-5

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