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Systemic Vasculitides: Current Status and Perspectives pp 129–139Cite as

Eosinophilic Granulomatosis with Polyangiitis (Churg-Straus Syndrome)

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Abstract

Eosinophilic Granulomatosis with Polyangiitis (EGPA) is defined as an eosinophil-rich and granulomatous inflammation involving the respiratory tract, and necrotizing vasculitis affecting small to medium-sized vessels, and associated with asthma and eosinophilia. It is usually classified among the so-called anti-neutrophil antibody (ANCA)-associated systemic vasculitides (AASVs) because of its clinical and pathologic features that overlap with those of the other AASVs. However, recent studies on large cohorts of patients have found that ANCA, usually P-ANCA/MPO-ANCA, were present in less than 40 % of patients. Moreover, ANCA status was shown to segregate with clinical phenotype. Preliminary results suggest that ANCA-positive and ANCA-negative patients also might have a different genetic background. Corticosteroids remain the cornerstone of the initial treatment of EGPA. The addition of cyclophosphamide is indicated in patients with poor-prognosis factors or in patients without poor-prognosis factors that relapse early. How long should maintenance therapy be continued remains to be established. However, the vast majority of patients require long-term corticosteroids treatment to control asthma.

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Author information

Authors and Affiliations

  1. Clinical Immunology Unit and Renal Unit, Azienda Ospedaliera Ospedale San Carlo Borromeo, Milano, Italy

    Renato Alberto Sinico

  2. Poliambulatorio Santa Crescenzia, Magenta, Milano, Italy

    Paolo Bottero

Authors
  1. Renato Alberto Sinico
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  2. Paolo Bottero
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Correspondence to Renato Alberto Sinico .

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Editors and Affiliations

  1. University of Bari Medical School, Bari, Italy

    Franco Dammacco

  2. University of Bari Medical School, Bari, Italy

    Domenico Ribatti

  3. University of Bari Medical School, Bari, Italy

    Angelo Vacca

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Sinico, R.A., Bottero, P. (2016). Eosinophilic Granulomatosis with Polyangiitis (Churg-Straus Syndrome). In: Dammacco, F., Ribatti, D., Vacca, A. (eds) Systemic Vasculitides: Current Status and Perspectives. Springer, Cham. https://doi.org/10.1007/978-3-319-40136-2_12

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