Severe eosinophilic granulomatosis with polyangiitis responding to a combination of rituximab and mepolizumab

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss Syndrome, is a multisystem antineu-trophil cytoplasmic antibody (ANCA) positive vasculitis, characterized by the presence of chronic rhinosinusitis, asthma and prominent peripheral blood eosinophilia. Although the most commonly involved organ is the lung, followed by the skin, EGPA can affect any organ system. Herein, we present the complicated case of an 18-year-old male patient with severe life-threat-ening EGPA, with central nervous system, cardiac and gasterointestinal involvement, which was resistant to initial treatment with glucocorticoids and cyclophosphamide. The patient responded well, achieving complete remission after the addition of rituximab and mepolizumab to glucocorticoids and cyclophosphamide.

Automated summary

Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystem antineutrophil cytoplasmic antibody (ANCA) positive vasculitis, characterized by chronic rhinosinusitis, asthma, and eosinophilia. This article presents the complex case of an 18-year-old male patient with severe and life-threatening EGPA, involving the central nervous system, heart, and gastrointestinal tract, which did not respond to initial glucocorticoid and cyclophosphamide treatment. The addition of rituximab and mepolizumab to the treatment regimen led to a good response and complete remission.