Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by eosinophilic vasculitis. Patients rarely present without asthma. Cases developing subarachnoid hemorrhage from central nervous system vasculitis are rarely reported. We report a 48-year-old woman with rapidly evolving and progressive multi-system eosinophilic vasculitis in the absence of asthma. Tissue eosinophilia was apparent in a breast lump biopsy. Prior otitis media and prominent lymphoid tissue in the postnasal spaces hinted at otolaryngological disease. She had rapid disease progression with mononeuritis multiplex and eventually succumbed to complications of intracranial hemorrhage secondary to central nervous system vasculitis. This case demonstrates the diagnostic dilemma and treatment considerations in EGPA without asthma. It also raises the question if a reliable biomarker can aid diagnosis in atypical presentations of disease.
Keywords: breast mass; eosinophilic granulomatosis with polyangiitis; subarachnoid hemorrhage.
© 2015 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.