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ANCA associated vasculitis

  • Fernando Salvador
    Affiliations
    Autoimmune Diseases Unit, Internal Medicine Department, Centro Hospitalar de Trás-os-Montes e Alto Douro, Avenida da Noruega, 5000-508 Vila Real, Portugal

    NEDAI/SPMI Autoimmune Diseases Group/Portuguese Society of Internal Medicine, Portugal
    Search for articles by this author
Published:January 29, 2020DOI:https://doi.org/10.1016/j.ejim.2020.01.011

      Highlights

      • Vasculitis is a multisystemic disease reaching vessels of different sizes.
      • Ideally, when suspected, a biopsy should confirm the diagnosis.
      • Treatment is based on two stages: induction and maintenance.
      • Glucocorticoids are generally used.
      • Methotrexate, cyclophosphamide, rituximab or azathioprine should also be considered.

      Abstract

      Vasculitis is characterized by inflammation of the vascular wall. It reaches vessels of different sizes and locations, conditioning multisystem and complex manifestations that require a holistic approach.
      Antineutrophil cytoplasmic antibody-associated vasculitis has an annual incidence rate of 20 per million inhabitants. It is the result of the interaction of infectious, genetic and environmental factors and manifest itself with varied and unspecific symptoms, often reaching the general state, respiratory and renal systems. The combination of clinical, laboratory, imaging and fundamentally histological changes allows the diagnosis and classification of the extent / severity of the disease necessary for the decision of the therapeutics to be taken. Glucocorticoids are generally used; with methotrexate being considered when localized disease and cyclophosphamide, rituximab or azathioprine when generalized disease.
      A systematized approach can increase survival rates from 12% to 70% over a 5-year span.

      Keywords

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