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静脉注射环磷酰胺和免疫球蛋白改善了一名嗜酸性肉芽肿性多血管炎患者的视野缺损。

Intravenous cyclophosphamide and immunoglobulin ameliorated visual field defects in a patient with eosinophilic granulomatosis with polyangiitis.

作者信息

Motobayashi Yuto, Oshikata Chiyako, Kodama Yuka, Terada Kosuke, Yamashita Yuga, Nakadegawa Ryo, Masumitsu Hinako, Osada Reeko, Takayasu Hirokazu, Masumoto Nami, Manabe Saki, Kaneko Takeshi, Shiraishi Akira, Tsurikisawa Naomi

机构信息

Department of Respirology, National Hospital Organization Yokohama Medical Center, 3-60-2 Harajuku, Totsuka-ku, Yokohama, 245-8575, Japan.

Department of Allergy and Respirology, Hiratsuka City Hospital, 1-19-1 Minamihara, Hiratsuka, Kanagawa, 254-0065, Japan.

出版信息

Respir Med Case Rep. 2024 Jan 9;47:101980. doi: 10.1016/j.rmcr.2024.101980. eCollection 2024.

Abstract

Treating ocular involvement in eosinophilic granulomatosis with polyangiitis (EGPA) can be challenging. We present the case of a 37-year-old woman with EGPA who had severe bilateral visual field defects. Laboratory results showed leukocytosis (17,500 WBC/μL, 25.8 % eosinophils), negative MPO-ANCA titer, and elevated PR3-ANCA level (33.2 IU/mL). Diffusion-weighted MRI revealed bilateral hyperintense occipital lesions, which were more prominent on the left. Her therapy initially included a steroid pulse, followed by daily prednisolone, but her visual field defects remained refractory. The addition of intravenous cyclophosphamide (5 courses) and intravenous immunoglobulin decreased her optic neuropathy and resolved her visual field defects.

摘要

治疗嗜酸性肉芽肿性多血管炎(EGPA)的眼部受累可能具有挑战性。我们报告了一例37岁患有EGPA的女性病例,她有严重的双侧视野缺损。实验室检查结果显示白细胞增多(白细胞计数17,500/μL,嗜酸性粒细胞25.8%),髓过氧化物酶抗中性粒细胞胞浆抗体(MPO-ANCA)滴度阴性,蛋白酶3抗中性粒细胞胞浆抗体(PR3-ANCA)水平升高(33.2 IU/mL)。弥散加权磁共振成像(MRI)显示双侧枕叶高信号病变,左侧更为明显。她的治疗最初包括一次类固醇冲击治疗,随后每日服用泼尼松龙,但她的视野缺损仍难以治疗。加用静脉注射环磷酰胺(5个疗程)和静脉注射免疫球蛋白后,她的视神经病变减轻,视野缺损得到改善。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6683/10827539/ad6f0e230f8a/gr1a.jpg

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