Internal Medicine Residency, Singapore city, Singapore.
Division of Rheumatology, University Medicine Cluster, National University Health System, Singapore city, Singapore.
Int J Rheum Dis. 2017 Dec;20(12):2127-2131. doi: 10.1111/1756-185X.12594. Epub 2015 May 11.
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.
嗜酸性肉芽肿伴多血管炎(EGPA)的特征为嗜酸性血管炎。患者很少没有哮喘而发病。因中枢神经系统血管炎而发生蛛网膜下腔出血的病例很少报道。我们报告一例 48 岁女性,表现为迅速进展和多系统嗜酸性血管炎,而无哮喘。乳腺肿块活检显示组织嗜酸性粒细胞增多。既往中耳炎和后鼻道淋巴组织增生提示耳鼻喉疾病。她有多发性单神经炎,并迅速进展,最终因中枢神经系统血管炎导致的颅内出血并发症而死亡。该病例表明了无哮喘的 EGPA 的诊断难题和治疗注意事项。它还提出了一个问题,如果有一种可靠的生物标志物能否帮助诊断疾病的非典型表现。