Takeuchi Eriko, Hirozawa Daisuke, Okiyama Naoko, Inoue Michio, Nishino Ichizo, Sugai Fuminobu
Department of Neurology, Otemae Hospital.
Present address: Department of Neurology and Cerebrovascular Disease, Osaka University Hospital.
Rinsho Shinkeigaku. 2021 Apr 21;61(4):258-261. doi: 10.5692/clinicalneurol.cn-001528. Epub 2021 Mar 25.
We report a 47-year-old woman who presented with progressive myalgia, weakness in the proximal limbs, and dysphagia for a month and a half. No skin rash was observed on admission. Examination of MRI data suggested inflammatory changes in the proximal limbs and trunk muscles. Biopsy specimens from the left biceps muscle showed no perifascicular atrophy, but immunohistochemical staining revealed the presence of myxovirus resistance protein A (MxA) in myofibers, strongly suggesting dermatomyositis (DM). In addition, her serum was positive for anti-nuclear matrix protein 2 (anti-NXP-2) antibody, which is reportedly useful as a marker of DM without skin lesions. Her symptoms gradually improved upon intravenous methylprednisolone pulse therapy in conjunction with oral prednisolone, oral tacrolimus, and intravenous immunoglobulin therapy. Our findings suggest that in cases where inflammatory muscle disease is suspected, anti-NXP-2 antibody analyses should be considered for precise diagnosis, even if there are no dermatological symptoms.
我们报告了一名47岁女性,她出现进行性肌痛、近端肢体无力及吞咽困难达一个半月。入院时未观察到皮疹。MRI数据检查提示近端肢体和躯干肌肉有炎症改变。左肱二头肌活检标本未显示束周萎缩,但免疫组化染色显示肌纤维中存在抗黏液病毒蛋白A(MxA),强烈提示皮肌炎(DM)。此外,她的血清抗核基质蛋白2(抗NXP - 2)抗体呈阳性,据报道该抗体可作为无皮肤病变的DM的标志物。经静脉注射甲泼尼龙冲击治疗联合口服泼尼松龙、口服他克莫司及静脉注射免疫球蛋白治疗后,她的症状逐渐改善。我们的研究结果表明,在怀疑有炎性肌病的病例中,即使没有皮肤症状,也应考虑进行抗NXP - 2抗体分析以进行精确诊断。