SP117CARDIOVASCULAR DISEASE IN EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS

Abstract

INTRODUCTION: Eosinophilic granulomatosis with polyangiitis (EGPA), is characterized by disseminated necrotising small vessel vasculitis with extravascular granulomas, amongst patients with the prodrome of asthma and tissue eosinophilia.1,2 The French Vasculitis Study Group five-factor prognostic score (FFS) associates cardiac disease with a poorer prognostic group.1,2,3 Histological findings in 7/9 cardiac transplant recipients, had evidence of EGPA in explanted native hearts despite ongoing immunosuppression.4 METHODS: This retrospective, descriptive study analysed 18 patients with EGPA according to the American College of Rheumatology criteria or Chapel Hill Consensus 2012 definition. Identification of cardiac disease was based upon abnormalities in clinical condition, cardiac enzymes, ECG, ECHO and in some cases cardiac MRI. The aim of our study was to analyse the clinical manifestation and outcomes of patients with cardiac disease. RESULTS: Themean age of onset of EGPA in 18 patientswas 47.8±11.1yrs. 100%were asthmatic. 15 wereANCA negative. At the time of mean follow-up 61.7±33.8months, percentage survival in the cohort was 100%. Two patients had evidence of thromboembolic disease. Pulmonary and ENT involvementwere common with cardiac disease, unlike renal disease. Cardiac manifestations and immunosuppressants are listed in table 1. CONCLUSIONS: In accordance with literature, cardiac disease was found predominantly in ANCA negative patients. Therapy with conventional immunosuppression including steroids, cyclophosphamide and biologic therapies rituximab, alemtuzumab and mepolizumab had a favourable outcome. Early diagnosis of cardiac involvement is essential in guiding management decisions and prognosis. (Table Presented) .