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

Abstract

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.

Anti-EJ antibody-positive interstitial pneumonia with breast cancer improved by combining immunosuppressive therapy and chemotherapy

“…Only three reports of malignancies with anti-EJ antibodies exist. These involved retroperitoneal sarcoma, rectal cancer, and nasopharyngeal cancer [ [12] , [13] , [14] ]. This reduced malignancy frequency in ASSD may correlate with IP presence, possibly reducing malignancy risk in DM patients [ 15 ].…”

Pericardial effusion in idiopathic inflammatory myopathies: A cross-sectional study from Asia and review of the literature

“…They included 1 (1/13, 7.7%), 8 (8/96, 8.3%), and 10 (10/271, 3.7%) patients, additionally adding five case reports, for a total of 25 patients. [13][14][15][16][17][18][19][20] Antisynthetase syndrome is described classically by the triad of interstitial lung disease, inflammatory myositis, and presence of aminoacyl-tRNA synthetase antibodies. In common with other reported IIM patients with pericardial effusion more prevalent in women, pulmonary fibrosis and positive anti-Jo-1 antibody were the most frequent manifestations, and these common features were confirmed in a review of all cases published to date in the literature.…”

Antisynthetase syndrome: A distinct disease spectrum

“…Other than the classic features mentioned above, calcinosis, panniculitis, coronary artery dilatation, eosinophilic pleural effusions, and myopericarditis have also been reported in small case series. [60][61][62][63][64][65][66][67][68][69]…”