Zeng Mingbing, Liu Xialin, Liu Yizhi
Zhongshang Ophthalmic Center, Sun Yat-sen University, Guangzhou, Guangdong, China (mainland).
Am J Case Rep. 2016 Nov 18;17:864-868. doi: 10.12659/ajcr.899441.
BACKGROUND Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome (CSS), is a rare vasculitis of unknown etiology. Most of the patients have a long history of asthma and then develop autoimmune inflammation of small and medium-sized blood vessels, with consequent reduction of blood flow to various organs and tissues. EGPA can cause disorders in multiple systems; the most seriously affected organs are the retina, kidney, brain, cardiovascular system, and skin. CASE REPORT The patient was hospitalized for high fever and skin rashes and then developed right upper abdominal pain, decreased visual acuity, coma, and convulsions. Laboratory investigations showed marked eosinophilia (9412/mm3). Following cholecystectomy, histopathological examination revealed a marked inflammatory cell infiltrate composed mainly of eosinophils. Retinal vasculitis and medium and peripheral vascular closure were confirmed by fundus fluorescence angiography (FFA). The coma and convulsions were controlled successfully by high-dose methylprednisolone. After gradual tapering of the methylprednisolone, the patient's blood count recovered to a normal level, and the other systematic disorders disappeared; however, she was left with irreversible blindness. CONCLUSIONS EGPA can cause eosinophilic cholecystitis, retinal vasculitis, and neuropathy in the short term and calls for effective treatments in order to avoid binocular blindness.
背景 嗜酸性肉芽肿性多血管炎（EGPA），又称变应性肉芽肿性血管炎（CSS），是一种病因不明的罕见血管炎。大多数患者有长期哮喘病史，随后发展为中小血管的自身免疫性炎症，导致各器官和组织血流减少。EGPA可引起多系统功能障碍；受影响最严重的器官是视网膜、肾脏、大脑、心血管系统和皮肤。病例报告 该患者因高热和皮疹入院，随后出现右上腹疼痛、视力下降、昏迷和惊厥。实验室检查显示明显的嗜酸性粒细胞增多（9412/mm³）。胆囊切除术后，组织病理学检查显示主要由嗜酸性粒细胞组成的明显炎性细胞浸润。眼底荧光血管造影（FFA）证实存在视网膜血管炎以及中周血管闭塞。大剂量甲泼尼龙成功控制了昏迷和惊厥。甲泼尼龙逐渐减量后，患者的血细胞计数恢复到正常水平，其他系统性疾病消失；然而，她仍遗留不可逆的失明。结论 EGPA可在短期内导致嗜酸性胆囊炎、视网膜血管炎和神经病变，需要进行有效治疗以避免双目失明。
