Terashima Seika, Yamaguchi Makoto, Ishihara Tomomi, Hagita Junichiro, Katsuno Takayuki, Ito Mayumi, Sugiyama Hirokazu, Iwagaitsu Shiho, Nobata Hironobu, Kinashi Hiroshi, Ishimoto Takuji, Banno Shogo, Ito Yasuhiko
Department of Nephrology and Rheumatology, Aichi Medical University, Nagakute, Japan.
Mod Rheumatol Case Rep. 2023 Jan 3;7(1):142-147. doi: 10.1093/mrcr/rxac073.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a type of anti-neutrophil cytoplasmic antibody-associated vasculitis characterised by small- to medium-sized vessel vasculitis and is typically associated with eosinophilic granulomatous inflammation. EGPA can affect any organ system, most commonly the lungs, skin, and the nervous system. However, limb ulcers are rare complications and have only been described in few case reports. Furthermore, no documented cases of EGPA have been treated with mepolizumab. Herein, we report a case of an 86-year-old Japanese woman with anti-neutrophil cytoplasmic antibody-negative EGPA, who had an abrupt onset of upper limb ulcers and bilateral foot drop due to multiple mononeuropathy. Clinicopathological sural nerve biopsy showed eosinophil-associated vascular damage. The patient was administered steroids, intravenous immunoglobulin, vasodilators, and mepolizumab; this resulted in clinical improvement of her finger ulcers and peripheral neuropathy without any adverse effects. In cases of an abrupt onset of limb ischaemia and peripheral neuropathy, physicians should consider the possibility of EGPA as a differential diagnosis. Furthermore, the early administration of mepolizumab might yield better outcomes in terms of improving limb ischaemia and peripheral neuropathy.
嗜酸性肉芽肿性多血管炎(EGPA)是一种抗中性粒细胞胞浆抗体相关性血管炎,其特征为中小血管血管炎,通常伴有嗜酸性肉芽肿性炎症。EGPA可累及任何器官系统,最常见的是肺、皮肤和神经系统。然而,肢体溃疡是罕见的并发症,仅有少数病例报告对此进行过描述。此外,尚无使用美泊利单抗治疗EGPA的文献记载病例。在此,我们报告一例86岁日本女性抗中性粒细胞胞浆抗体阴性的EGPA患者,该患者因多发性单神经病突然出现上肢溃疡和双侧足下垂。临床病理腓肠神经活检显示嗜酸性粒细胞相关性血管损伤。给予该患者类固醇、静脉注射免疫球蛋白、血管扩张剂和美泊利单抗治疗;这使得她的手指溃疡和周围神经病变得到临床改善,且未出现任何不良反应。对于突然出现肢体缺血和周围神经病变的病例,医生应考虑EGPA作为鉴别诊断的可能性。此外,早期使用美泊利单抗在改善肢体缺血和周围神经病变方面可能会产生更好的效果。