Antisynthetases syndrome associated with right heart failure

Area of Science:

• Rheumatology
• Cardiology
• Neurology

Background:

• Cardiac involvement is a known complication of polymyositis, typically presenting as left heart insufficiency.
• Antisynthetase syndrome is an autoimmune disorder characterized by autoantibodies targeting synthetase enzymes, often associated with polymyositis and interstitial lung disease.

Observation:

• A 26-year-old male presented with a 6-month history of polyarthritis, fever, myalgia, and elevated inflammatory markers.
• Initial cardiac evaluations including echocardiography were normal, but the patient acutely developed right heart failure.
• Magnetic resonance imaging revealed right ventricular myocarditis with inflammatory thickening.

Findings:

• The patient tested positive for anti-SSA and anti-JO1 antibodies, alongside evidence of myolysis and a myopathic pattern on electromyography.
• Muscle biopsy confirmed polymyositis, a component of the antisynthetase syndrome.
• Despite normal initial cardiac imaging, the patient developed significant right-sided heart failure due to acute myocarditis.

Implications:

• This case underscores the importance of considering cardiac involvement, particularly right-sided heart failure, in antisynthetase syndrome, even with normal initial cardiac assessments.
• Early recognition and treatment with corticosteroids can lead to rapid improvement in both clinical symptoms and biological markers.
• This presentation expands the spectrum of cardiac manifestations in antisynthetase syndrome, emphasizing the need for comprehensive cardiovascular evaluation in affected individuals.