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Eosinophilic Granulomatosis with Polyangiitis (EGPA, Churg–Strauss)

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Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis

Part of the book series: Rare Diseases of the Immune System ((RDIS))

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Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg–Strauss syndrome, a systemic necrotizing vasculitis of small- and medium-sized vessels, is characterized by asthma and blood eosinophilia, and anti-myeloperoxidase (MPO) anti-neutrophil cytoplasm antibodies (ANCA) in one-third of the patients. Its pathophysiology is complex, with several mechanisms being involved. EGPA typically occurs in previously asthmatic patients, and involves the skin, lungs, and peripheral nerves. Involvements of the heart, gastrointestinal tract, and/or kidney are severe, and associated with high mortality, as assessed by the Five-Factor Score (FFS). Overall survival has improved dramatically since corticosteroids and immunosuppressants have been prescribed, but relapses remain frequent. ANCA status seems to differentiate two groups with different clinical features and prognoses. All patients require corticosteroids, combined with an immunosuppressant (cyclophosphamide for induction and azathioprine for maintenance therapy) for those with severe/refractory disease and FFS-defined poor prognoses. Recent advances in EGPA management, including several novel immunomodulatory drugs and biotherapies, were or are being evaluated to improve EGPA patients’ prognoses.

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Authors and Affiliations

  1. Department of Internal Medicine, Referral Center for Rare Systemic and Autoimmune Diseases, Hôpital Cochin, AP-HP, Université Paris Descartes, Paris, France

    Yann Nguyen & Loïc Guillevin

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  1. Yann Nguyen
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  2. Loïc Guillevin
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  1. Department of Medicine and Surgery, University of Milano-Bicocca, Monza, Monza e Brianza, Italy

    Renato Alberto Sinico

  2. Department of Medicine, Paris Descartes University Hospital Cochin, Paris, France

    Loïc Guillevin

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Nguyen, Y., Guillevin, L. (2020). Eosinophilic Granulomatosis with Polyangiitis (EGPA, Churg–Strauss). In: Sinico, R., Guillevin, L. (eds) Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis. Rare Diseases of the Immune System. Springer, Cham. https://doi.org/10.1007/978-3-030-02239-6_6

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