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Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): A Clinical and Therapeutic Approach

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Abstract

Churg–Strauss syndrome (CSS), first described in 1951, is a rare small- and medium-sized–vessel vasculitis characterized by the constant association with asthma and eosinophilia, and anti-myeloperoxidase (MPO) antineutrophil cytoplasm antibodies (ANCA) in ~40 % of the patients. Vasculitis typically develops in a previously asthmatic and eosinophilic middle-aged patient, and most frequently involves the peripheral nerves and skin. Other organs, however, may be affected and must be screened for vasculitis, especially those associated with a poorer prognosis, e.g., the heart, kidney and gastrointestinal tract. Overall survival of CSS patients is excellent but relapses are not uncommon and require maintenance or steroid-sparing therapies. All patients require corticosteroids, often for prolonged periods, combined with immunosuppressants (e.g., induction (cyclophosphamide) and maintenance therapy (azathioprine)), for those with poorer prognoses. Recent insights, especially concerning clinical differences associated with ANCA status, suggested that CSS patients might constitute a clinically and pathogenically heterogeneous group. Future therapies might perhaps reflect these differences more strongly.

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  1. Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases (Including Vasculitis, Scleroderma), INSERM U1060, Hôpital Cochin, Assistance Publique–Hôpitaux de Paris, Université Paris Descartes, 27, rue du faubourg Saint-Jacques, Paris, 75679, France

    Loïc Guillevin MD & Matthieu Groh MD

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  1. Loïc Guillevin MD
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  2. Matthieu Groh MD
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Correspondence to Loïc Guillevin MD or Matthieu Groh MD .

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  1. Rare Pulmonary Diseases, Hôpital Louis Pradel, Lyon, France

    Vincent Cottin

  2. Rare Pulmonary Diseases, Hôpital Louis Pradel, Lyon, France

    Jean-Francois Cordier

  3. Respiratory Medicine, University of Southampton, Southampton, United Kingdom

    Luca Richeldi

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Guillevin, L., Groh, M. (2015). Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): A Clinical and Therapeutic Approach. In: Cottin, V., Cordier, JF., Richeldi, L. (eds) Orphan Lung Diseases. Springer, London. https://doi.org/10.1007/978-1-4471-2401-6_8

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