Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss Syndrome, is a multisystem antineutrophil 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-threatening 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.
Eosinophilic granulomatosis with polyangiitis (EGPA), is an eosinophilic necrotizing vasculitis of small and medium sized blood vessels, which often presents as a multisystem disease, mainly affecting the lungs, skin and peripheral nervous system. Asthma is the cardinal clinical feature of the disease, which together with eosinophilia may precede the diagnosis of EGPA.
Antineutrophil cytoplasmic antibodies (ANCA) are present in 30–40% of the patients and are almost all against myeloperoxidase (MPO) than proteinase 3 (PR3). Herein, we present a case of a young male patient with EGPA, with severe central nervous system involvement (CNS) as well as cardiac disease, who responded to the administration of rituximab and mepolizumab, as the disease was resistant to the conventional treatment with glucocorticoids and cyclophosphamide.