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

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.