DOI: 10.36347/sjmcr.2019.v07i02.014

Pages: 149-154

Background: The antisynthetase syndrome (ASS) is characterized by autoimmune myopathy, interstitial lung disease, cutaneous involvement, arthritis, fever, and antibody specificity. Clinicians should be familiar with this entity. Cardiac involvement is uncommon but possible. Case report: We describe anti PL12 positive patient with antisynthetase syndrome who also developed complete atrioventricular heart block, depressed biventricular function leading to congestive heart failure and pulmonary hypertension. The patient was diagnosed with ASS based on clinical manifestations, detection of serum AS antibodies, myositis confirmation and presence of diffuse interstitial lung involvement. She was treated with diuretic and corticosteroid therapy. Permanent pacemaker implantation was performed with no complication. On the follow up, the patient responded well to steroids and cyclophosphamide pulse. Conclusion: Antisynthetase syndrome is a rare entity with morbidity and mortality typically attributed to myositis and lung involvement. This is the first report of ASS associated complete heart block, cardiomyopathy and pulmonary hypertension. Given the potentially fatal consequences, the cardiac involvement should be considered in patients with antisynthetase syndrome.