Rinsho Shinkeigaku (Clinical Neurology)

Case Report

A case of antisynthetase syndrome with anti-EJ antibody complicated by pericarditis

Kumiko Hayashi, M.D.1), Yoko Machida, M.D.1), Yuki Katayama, M.D.1), Hiroaki Yokote, M.D., Ph.D.1), Kazuyuki Saito, M.D., Ph.D.1), Mayumi Masumura, M.D.2), Akiko Miyashita, M.D.3), Masaki Kobayashi, M.D., Ph.D.3)4) and Shuta Toru, M.D., Ph.D.1)

1)Department of Neurology, Nitobe Memorial Nakano General Hospital
2)Department of Internal Medicine, Nitobe Memorial Nakano General Hospital
3)Department of Neurology and Neurological Science, Tokyo Medical and Dental University
4)Department of Neurology, Yokufukai Hospital

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.
Full Text of this Article in Japanese PDF (742K)

(CLINICA NEUROL, 59: 21|26, 2019)
key words: anti-EJ antibody, antisynthetase syndrome, pericarditis, interstitial pneumonia, myositis

(Received: 4-Feb-18)