278. USE OF RITUXIMAB AND MEPOLIZUMAB FOR EGPA

Abstract

Background: Eosinophilic Granulomatosis with Polyangiitis (EGPA) formally known as Churg-Strauss Syndrome has both allergic and vasculitic components. Two major therapeutic modalities for these attributes have been recently utilized - Rituximab for the vasculitic component and mepolizumab for the allergic component. Case Report:N.P. was a 17 year old male at presentation in 2015 with a two year history of chronic sinusitis, asthma, eosinophilia, pulmonary infiltrates, elevated inflammatory markers, elevated IgE level and known allergy to multiple pollens and molds. The syndrome was poorly controlled on inhalers and allergy shots with development of nasal polyps that were removed and the pathology on the tissue was nonspecific. Pulmonary infiltrates with ground glass opacities SkWMn r adenop athy required systemic steroids. Later he developed a rash and a pericardial e p T usion that responded to 60 mg of prednisone a day but flared when the dose was reduced to 30mg/day. Bronchoscopy showed purulent secretions but cultures were unremarkable. An ANCA test was negative. Subsequently he was started on rituximab and he was able to reduce his prednisone requirement to 10 mg a day but still required several burst and tapers of prednisone and eventually daily prednisone to 20mgin spite of xopenex and inhalers. Daily Bactrim was modestly helpful but he developed rash and fever c/w a sulfa reaction and it was stopped. After 1 year on Q4 months rituximab we added mepolizumab at 300mg Q 28 days and an allergist added singular, Flonase and azelastine which reduced his steroid requirement to 15mg a day. Most recently his IgE level remains elevated at 142 but his IL-5 level is undetectable, his CD 19 count is 0 ESR 3 and CRP 3.5 with an overall improvement in his functionality. No toxicity was noted on the combination of rituximab and mepolizumab. This case report suggests that both agents can be used together in the same patient. [Figure Presented].