Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare type of anti-neutrophil cytoplasm antibody-associated vasculitis (AAV) with unique features, such as involvement of eosinophils in the pathogenesis, which requires different therapies from those used for other AAV. Conventional treatment includes glucocorticoids (GC) and immunosuppressants. GC are the cornerstone of the initial treatment of EGPA, but relapses are frequent. Cyclophosphamide is typically used in combination with GC for patients with life- and/or organ-threatening disease manifestations. Azathioprine and methotrexate are recommended to maintain remission after induction with cyclophosphamide or as a GC-sparing agent. Nowadays, a better comprehension of the physiopathology of EGPA has opened new therapeutic targets, such as interleukin-5, which has a key role in the refractory disease, relapses, and GC dependence, especially for asthma manifestations. Mepolizumab is the first anti-IL5 antibody approved to treat EGPA. Another anti-IL5 monoclonal antibody, reslizumab, and an anti-IL5 receptor monoclonal antibody, benralizumab, are now being investigated for EGPA.
Part of the book: Cells of the Immune System