Antisynthetase syndrome (AS) is a rare systemic autoimmune disease characterized by the presence of anti-tRNA synthetase antibodies. Cardiac involvement (pericarditis) in AS is uncommon. Here, we present the case of a young African female who presented with acute pericarditis as an initial manifestation of AS. She was diagnosed with anti-Jo1 antisynthetase syndrome based on Connors criteria. Treatment with corticosteroids and azathioprine improved symptoms. This case underlines the importance for clinicians to evoke the possibility of an antisynthetase syndrome in the case of acute pericarditis.
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