Eosinophilic granulomatosis with polyangiitis: Pathogenesis and therapeutic consequences

Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss) is a rare small sized vessel vasculitis associated with asthma and blood and tissue eosinophilia. EGPA belongs to the group of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, although ANCA are present in only one third of patients. EGPA shares some similarities with asthma, eosinophilic disorders and other ANCA-associated vasculitides. Mechanisms are however still unknown, explaining why therapeutic management is mainly based on nonspecific treatments, such as steroids and conventional immunosuppressants. Biological therapies are under evaluation, primarily antibodies targeting interleukin-5 and its receptor (mepolizumab, benralizumab), and rituximab targeting B cells. (C) 2019 l'Academie nationale de medecine. Published by Elsevier Masson SAS. All rights reserved.