Desmoplakin Cardiomyopathy Presenting With Recurrent Myopericarditis Responsive to Interleukin-1 Blockade

Acute myopericarditis in patients with pathogenic desmoplakin (DSP) variants are associated with a high risk for heart failure and cardiac arrhythmias. We present the case of a 25-year-old woman who presented with recurrent myopericarditis with chest pain and elevated troponin I levels despite escalating prednisone therapy. Genetic testing revealed a pathogenic variant in DSP along with a pathogenic variant in KCNQ1 associated with long-QT syndrome and a NOD2 variant associated with an increased risk for Crohn disease. A subcutaneous implantable cardioverter-defibrillator was implanted. The patient was started on the interleukin-1 inhibitor rilonacept, resulting in a significant reduction in symptoms and troponin I levels and the ability to wean off prednisone. Nine months after initiating rilonacept, the patient remained asymptomatic, had been off prednisone for 8 months, and demonstrated improved exercise tolerance and normalized troponin I levels. This case highlights the potential benefit of interleukin-1 blockade in managing DSP-related recurrent myopericarditis.