Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) Medication

Updated: Dec 24, 2018
  • Author: Spencer T Lowe, MD; Chief Editor: Herbert S Diamond, MD  more...
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Medication

Medication Summary

Glucocorticoids are the cornerstone of therapy for eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss syndrome). [2] For life- or organ-threatening disease, glucocorticoids are combined with other immunosuppressant drugs, such as cyclophosphamide, for induction of remission, and maintenance therapy is provided with azathioprine or methotrexate. [2]

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Corticosteroids

Class Summary

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the immune response to diverse stimuli.

Prednisone (Deltasone, Orasone, Sterapred)

Immunosuppressant for treatment of autoimmune disorders. May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Oral prednisone is usually sufficient to control disease activity.

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Cytotoxic agents

Class Summary

These agents inhibit cell growth and proliferation. They are reserved for cases resistant to corticosteroids.

Cyclophosphamide (Cytoxan, Neosar)

Chemically related to nitrogen mustards. As an alkylating agent, the mechanism of action of the active metabolites may involve cross-linking of DNA, which interferes with growth of rapidly proliferating cells.

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Monoclonal Antibodies, Anti-asthmatics

Class Summary

Eosinophils produce proinflammatory mediators, such as eosinophilic cationic protein (ECP) and leukotrienes. IL-5 promotes eosinophil differentiation and activation, as well as trafficking into the lungs. Monoclonal antibodies against IL-5 have been developed to target eosinophilic inflammation.

Mepolizumab (Nucala)

Humanized IgG1 kappa monoclonal antibody specific for IL-5; binds IL-5, and therefore stops IL-5 from binding to its receptor on the surface of eosinophils. By inhibiting IL-5 signaling, mepolizumab reduces the production and survival of eosinophils.

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