ANCA negative eosinophilic granulomatosis with polyangiitis: sometimes it really IS vasculitis

Abstract

SUMMARYA 21-year-old woman, with a background of asthma, presented to medical admissions ward, with diarrhoea and vomiting; the clinical picture during her admission evolved to include acute shortness of breath, seizures, unsteadiness, low mood and apathy. Investigations revealed pericardial, pleural effusions, myocardial infiltration and vasculitic lesions in the brain. Although serological tests for autoantibodies were negative, an eventual diagnosis of eosinophilic granulomatosis with polyangiitis, formerly known as Churg-Strauss syndrome, was performed based on the clinical picture. The multiorgan involvement meant that the initial diagnosis and effective management required multidisciplinary input from cardiology, neurology, rheumatology, psychiatry, immunology and occupational and physiotherapy. BACKGROUND

Cardiac Involvement in Eosinophilic Granulomatosis with Polyangiitis: A Meta-analysis of 62 Case Reports

“…The CNS involvement, as well as the kidney and GI involvement, was not a common presentation in our study population. The CNS vasculitis presented with ischemic brain lesions, 15 , 39 , 55 subarachnoid hemorrhage, 54 seizure, 36 or rarely with oculomotor nerve palsy. 16 In a case reported by Bujak et al, 45 the patient presented with the CNS symptoms due to brain ischemia but it was actually due to embolization from an LV thrombus.…”