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]The monoclonal antibody mepolizumab is approved for use in EGPA. Rituximab and omalizumab are often used off-label, especially in steroid-refractory or relapsing cases.

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)

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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.

Class Summary

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

Cyclophosphamide (Cytoxan, Neosar)

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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.

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)

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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.

Questions

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Media Gallery

Transient pulmonary infiltrates in a patient with Churg-Strauss syndrome (CSS).
Eosinophilic granuloma in a patient with Churg-Strauss syndrome (CSS).
The skin rashes of Churg-Strauss syndrome. The biopsy of this rash showed eosinophilic leukocytoclastic angiitis with poorly formed granulomas.

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Author

Spencer T Lowe, MD Rheumatologist, Private Practice, Peninsula Medical Group, Burlingame, CA

Spencer T Lowe, MD is a member of the following medical societies: American College of Rheumatology, California Medical Association, International Society for Clinical Densitometry

Disclosure: Nothing to disclose.

Coauthor(s)

Christopher L Tracy, MD Associate Program Director, Rheumatology Fellowship Program, Walter Reed National Military Medical Center

Christopher L Tracy, MD is a member of the following medical societies: American College of Physicians, American College of Rheumatology, American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Lawrence H Brent, MD Associate Professor of Medicine, Sidney Kimmel Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, American College of Rheumatology

Disclosure: Stock ownership for: Johnson & Johnson.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

Additional Contributors

Mehran Farid-Moayer, MD Adjunct Clinical Faculty, Department of Psychiatry, Sleep Disorders Clinic, Stanford Medical Center

Mehran Farid-Moayer, MD is a member of the following medical societies: American Academy of Sleep Medicine, American Medical Association

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous coauthor, Sandra L Sessoms, MD, to the development and writing of this article.