Catastrophic subarachnoid hemorrhage in eosinophilic granulomatosis with polyangiitis without asthma

• Published in: International journal of rheumatic diseases Vol. 20; no. 12; pp. 2127 - 2131
• Main Authors: Lee, Matilda X. W., Teng, Gim Gee, Raju, Gangaraju Changal, Lim, Anita Y. N.
• Format: Journal Article
• Language: English
• Published: England Wiley Subscription Services, Inc 01.12.2017
• Subjects: Asthma; Biopsy; breast mass; Central nervous system; Eosinophilia; eosinophilic granulomatosis with polyangiitis; Granulomatosis; Hemorrhage; Inflammatory diseases; Leukocytes (eosinophilic); Lymphoid tissue; Nervous system; Otitis media; Subarachnoid hemorrhage; Vasculitis; Vein & artery diseases; breast mass; eosinophilic granulomatosis with polyangiitis; subarachnoid hemorrhage
• ISSN: 1756-1841; 1756-185X
• DOI: 10.1111/1756-185X.12594

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.