Najem Catherine E, Yadav Rajwardhan, Carlson Elise
Department of Internal Medicine, Roger Williams Medical Center, Providence, Rhode Island, USA.
Department of Rheumatic and Immunologic Diseases, Yale New Haven Hospital, New Haven, Connecticut, USA.
BMJ Case Rep. 2015 May 15;2015:bcr2014206421. doi: 10.1136/bcr-2014-206421.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare eosinophil-rich disorder characterised by necrotising granulomatous inflammation affecting small to medium sized vessels. Extrapulmonary manifestations can be life-threatening when heart, central nervous system (CNS), gastrointestinal tract or kidneys are affected. We describe a case of a 56-year-old woman with a long-standing history of asthma, who presented with an acute sudden painless loss of vision after she had been recently diagnosed with EGPA and induced with pulse steroids and cyclophosphamide. The patient had a complicated hospital course with multisystemic involvement of active vasculitis, involving heart, kidneys, muscles, eyes and CNS. The patient's devastating condition responded remarkably to Rituximab. The role of Rituximab in EGPA is not yet proven. Few cases are reported in the literature about the role of Rituximab in EGPA, of which only one described retinal artery occlusion as a presentation of a recently treated patient with EGPA.
嗜酸性肉芽肿性多血管炎（EGPA）是一种罕见的富含嗜酸性粒细胞的疾病，其特征为影响中小血管的坏死性肉芽肿性炎症。当心脏、中枢神经系统（CNS）、胃肠道或肾脏受累时，肺外表现可能危及生命。我们描述了一例56岁女性患者，她有长期哮喘病史，在最近被诊断为EGPA并接受脉冲类固醇和环磷酰胺诱导治疗后，出现急性无痛性视力丧失。该患者住院过程复杂，有多系统活动性血管炎累及，包括心脏、肾脏、肌肉、眼睛和中枢神经系统。患者的严重病情对利妥昔单抗反应显著。利妥昔单抗在EGPA中的作用尚未得到证实。文献中很少有关于利妥昔单抗在EGPA中作用的报道，其中仅有一例描述了视网膜动脉阻塞是一名近期接受治疗的EGPA患者的表现。
