Hayashi Kumiko, Machida Yoko, Katayama Yuki, Yokote Hiroaki, Saito Kazuyuki, Masumura Mayumi, Miyashita Akiko, Kobayashi Masaki, Toru Shuta
Department of Neurology, Nitobe Memorial Nakano General Hospital.
Department of Internal Medicine, Nitobe Memorial Nakano General Hospital.
Rinsho Shinkeigaku. 2019 Jan 30;59(1):21-26. doi: 10.5692/clinicalneurol.cn-001140. Epub 2018 Dec 29.
A 69-year-old man was admitted with neck muscle weakness, symmetric proximal muscle weakness, skin rash and elevated serum creatine kinase levels. Muscle biopsy showed perifascicular necrosis and perimysial alkaline phosphatase activity. Chest CT revealed interstitial lung disease and colorectal cancer was diagnosed on colonoscopy. He was serologically positive for anti-EJ antibody, leading to the diagnosis of antisynthetase syndrome (ASS). After laparoscopic low anterior resection of the rectum, he received intravenous methylprednisolone (1,000 mg/d for 3 days) followed by oral prednisolone (50 mg/d). Although his muscle weakness improved after corticosteroid therapy, he developed pericardial effusion with resultant asymptomatic hypotension and arrhythmia possibly due to pericarditis. Corticosteroid monotherapy was insufficient to control the disease, and, we decided to use oral cyclosporin concurrently. After this combined therapy started, pericardial effusion and arrhythmia were improved. We should keep in mind that pericarditis can occur in patients with anti-EJ antibody-positive ASS, and early combined therapy with corticosteroid and immunosuppressive drugs for ASS may improve the patient's prognosis.
一名69岁男性因颈部肌肉无力、对称性近端肌肉无力、皮疹及血清肌酸激酶水平升高入院。肌肉活检显示束周坏死和肌周碱性磷酸酶活性。胸部CT显示间质性肺疾病，结肠镜检查诊断为结直肠癌。他抗EJ抗体血清学检测呈阳性，诊断为抗合成酶综合征（ASS）。直肠腹腔镜低位前切除术后，他接受了静脉注射甲泼尼龙（1000mg/d，共3天），随后口服泼尼松龙（50mg/d）。尽管皮质类固醇治疗后他的肌肉无力有所改善，但他出现了心包积液，导致无症状性低血压和心律失常，可能是由于心包炎所致。单纯皮质类固醇治疗不足以控制病情，因此我们决定同时使用口服环孢素。开始这种联合治疗后，心包积液和心律失常得到改善。我们应牢记，抗EJ抗体阳性的ASS患者可能会发生心包炎，早期对ASS采用皮质类固醇和免疫抑制药物联合治疗可能会改善患者的预后。
