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成功地早期引入美泊利珠单抗治疗髓过氧化物酶抗中性粒细胞胞质抗体阴性嗜酸粒细胞肉芽肿伴多血管炎伴周围神经病变伴周围循环障碍患者。

Successful early introduction of mepolizumab for peripheral neuropathy with a peripheral circulatory disorder in a patient with myeloperoxidase anti-neutrophil cytoplasmic antibody-negative eosinophilic granulomatosis with polyangiitis.

机构信息

Division of Hematology and Rheumatology, Department of Medicine, Nihon University School of Medicine, Tokyo, Japan.

出版信息

Mod Rheumatol Case Rep. 2021 Jul;5(2):354-359. doi: 10.1080/24725625.2021.1916159. Epub 2021 May 10.

Abstract

A 26-year-old woman presented with abdominal pain, diarrhoea, vomiting, fever, and progressive paralysis in the lower limbs. She had a history of bronchial asthma and experienced sinusitis, progressive peripheral neuropathy, polyarthritis, and leukocytosis with prominent eosinophilia. The patient was diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA). Abdominal pain was considered to be an ischaemic enteritis associated with EGPA. She was administered 1,000 mg/day of methylprednisolone for 3 days and intravenous immunoglobulin (400 mg/kg/day of γ-globulin for 5 days) followed by 50 mg (1 mg/kg)/day of oral prednisolone due to rapidly progressing peripheral neuropathy. Her symptoms temporarily improved; however, peripheral neuropathy recurred after a week, and the eosinophil count increased. Eighteen days after following the resumed treatment, 300 mg of mepolizumab, a humanised monoclonal antibody, was administered. Subjective symptoms, nerve conduction velocity, and skin perfusion pressure (an index of peripheral circulation in the lower extremities) improved after 4 weeks. Although mepolizumab has been approved for EGPA, there is no evidence of its efficacy against peripheral neuropathy. Early introduction of mepolizumab may contribute to an the early improved progressive peripheral neuropathy with eosinophilia.

摘要

一位 26 岁女性因腹痛、腹泻、呕吐、发热和下肢进行性瘫痪就诊。她有支气管哮喘病史,曾患有鼻窦炎、进行性周围神经病、多发性关节炎和白细胞增多伴明显嗜酸性粒细胞增多。患者被诊断为嗜酸性肉芽肿性多血管炎(EGPA)。腹痛被认为是与 EGPA 相关的缺血性肠炎。由于迅速进展的周围神经病,她接受了 1000mg/天的甲基强的松龙治疗 3 天和静脉注射免疫球蛋白(400mg/kg/天γ球蛋白 5 天),然后口服泼尼松 50mg(1mg/kg/天)。她的症状暂时有所改善;然而,一周后周围神经病再次发作,嗜酸性粒细胞计数增加。在恢复治疗后 18 天,给予了 300mg 的美泊利珠单抗,一种人源化单克隆抗体。主观症状、神经传导速度和皮肤灌注压(下肢外周循环的指标)在 4 周后得到改善。尽管美泊利珠单抗已被批准用于 EGPA,但尚无其对周围神经病疗效的证据。早期引入美泊利珠单抗可能有助于改善伴有嗜酸性粒细胞增多的进行性周围神经病。

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