Key result
Steroid tapering precipitates fatal cardiogenic shock after initial IVIG response in anti-Kv1.4 myocarditis.
Why the study?
Anti-Kv1.4 antibody-associated myocarditis is an extremely rare condition, and its optimal long-term management remains unclear.
Case Report (n=1)
No
This case report highlights that anti-Kv1.4 antibody-associated myocarditis can follow a fatal course with progressive ventricular dysfunction and cardiogenic shock during steroid tapering despite an initial favorable response to immunosuppressive therapy.
Answers are grounded in this paper and cite their sources.
May support IVIG plus corticosteroids for acute anti-Kv1.4 myocarditis; leaves open optimal long-term management.
Background Anti-Kv1.4 antibody-associated myocarditis is an extremely rare condition and its optimal long-term management remains unclear. Case Summary A 66-year-old woman presented with myasthenia gravis-like symptoms, marked creatine kinase elevation, leukopenia, and hypogammaglobulinaemia. She developed acute myocarditis with ventricular arrhythmias and a reduced left ventricular ejection fraction. Intravenous immunoglobulin and corticosteroid therapy resulted in rapid clinical and cardiac improvement. During cautious tapering of oral prednisolone, she was readmitted with febrile neutropenia and subsequently developed progressive ventricular dysfunction and cardiogenic shock. Cardiac magnetic resonance imaging demonstrated extensive myocardial fibrosis, and autopsy revealed diffuse lymphocytic infiltration. Postmortem testing confirmed positivity for anti-Kv1.4 antibodies. Discussion This case highlights the potentially fatal course of suspected anti-Kv1.4 antibody-associated inflammatory myocardial disease despite an initial favourable response to immunosuppressive therapy. It illustrates the diagnostic and therapeutic challenges of distinguishing immune-mediated progression from other causes of myocardial injury and implies the need for individualised long-term management strategies.
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Kato et al. (2026) conducted a case report in Anti-Kv1.4 antibody-associated myocarditis (n=1). Intravenous immunoglobulin and corticosteroid therapy (including oral prednisolone) was evaluated on Clinical and cardiac progression (ventricular dysfunction, cardiogenic shock, and death). Intravenous immunoglobulin and corticosteroids produced initial improvement in a 66-year-old woman with anti-Kv1.4 myocarditis, but prednisolone tapering led to fatal cardiogenic shock.
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