Successful use of Rituximab in a patient with recalcitrant multisystemic eosinophilic granulomatosis with polyangiitis

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare eosinophil-rich disorder characterised by necrotising granulomatous inflammation affecting small to medium sized vessels. Extrapulmonary manifestations can be life-threatening when heart, central nervous system (CNS), gastrointestinal tract or kidneys are affected. We describe a case of a 56-year-old woman with a long-standing history of asthma, who presented with an acute sudden painless loss of vision after she had been recently diagnosed with EGPA and induced with pulse steroids and cyclophosphamide. The patient had a complicated hospital course with multisystemic involvement of active vasculitis, involving heart, kidneys, muscles, eyes and CNS. The patient's devastating condition responded remarkably to Rituximab. The role of Rituximab in EGPA is not yet proven. Few cases are reported in the literature about the role of Rituximab in EGPA, of which only one described retinal artery occlusion as a presentation of a recently treated patient with EGPA.