Pharmacological approach to treating eosinophilic granulomatosis with polyangiitis (Churg–Strauss)

Abstract

Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA), formerly named Churg–Strauss syndrome, is an antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) occurring in asthmatic patients. EGPA prognosis has been revolutionized by the use of glucocorticoids and immunosuppressant(s). Following recent advances in the management of AAV, eosinophilic disorders and asthma, several novel immunomodulatory drugs and biotherapies were or are currently being evaluated for EGPA.

Areas covered: Herein, based on pathogenesis and clinical characteristics, we review EGPA standard of care; the different therapeutic options available; and address forthcoming therapeutic perspectives.

Expert opinion: EGPA prognosis is very good, with overall 5-year survival now reaching 90%. Like other systemic necrotizing vasculitides, EGPA treatment comprises induction and maintenance phases. Glucocorticoids are the cornerstone of induction therapy. Cyclophosphamide should be added when the prognosis is poor (as defined by the prognostic Five-Factor Score). Immunosuppressants (e.g., mainly azathioprine or methotrexate) are prescribed for an as yet undefined time (usually 18 – 24 months) following remission-induction. Chronic asthma, peripheral neuropathy, heart involvement and glucocorticoid-dependence are challenging clinical situations. Biotherapies (e.g., omalizumab, rituximab and mepolizumab) hold promise for EGPA treatment. Whether patients might benefit from distinct therapeutic strategies depending on their ANCA status (e.g., rituximab for ANCA-positive or mepolizumab for ANCA-negative patients) requires further investigation.

Keywords:

• biotherapies
• eosinophilia
• eosinophilic granulomatosis with polyangiitis
• glucocorticoids
• immunosuppressants
• systemic necrotizing vasculitides

Acknowledgement

The authors thank Mrs Janet Jacobson for editorial assistance.

Declaration of interest

M Groh has received congress registration funded by LFB Biotechnologies and Octapharma. B Dunogue is an investigator for an international clinical trial on mepolizumab for EGPA. B Dunogue has also received congress registration funded by LFB Biotechnologies and GSK. L Guillevin has received grants from Assistance Publique-Hopitaux De Paris for prospective trials in ANCA-associated vasculitis, as well as personal fees from Roche, GSK, Pfizer, Actelion, CSL Behring and LFB Biotechnologies for lectures and board meetings outside of the submitted work. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Notes

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