Abstract

Support measures currently represent the mainstay of treatment for fulminant myocarditis, while effective and safe anti-inflammatory therapies remain an unmet clinical need. However, clinical and experimental evidence indicates that inhibition of the pro-inflammatory cytokine interleukin 1 (IL-1) is effective against both myocardial inflammation and contractile dysfunction. We thus evaluated treatment with the IL-1 receptor antagonist anakinra in a case of heart failure secondary to fulminant myocarditis. A 65-year-old man with T cell lymphoma developed fulminant myocarditis presenting with severe biventricular failure and cardiogenic shock requiring admittance to the intensive care unit and mechanical circulatory and respiratory support. Specifically, acute heart failure and cardiogenic shock were initially treated with non-invasive ventilation and mechanical circulatory support with an intra-aortic balloon pump. Nevertheless, cardiac function deteriorated further, and there were no signs of improvement. Treatment with anakinra, the recombinant form of the naturally occurring IL-1 receptor antagonist, was started at a standard subcutaneous dose of 100 mg/day. We observed a dramatic clinical improvement within 24 h of initiating anakinra. Prompt, progressive amelioration of cardiac function allowed weaning from mechanical circulatory and respiratory support within 72 h of anakinra administration. Recent studies point at inhibition of IL-1 activity as an attractive treatment option for both myocardial inflammation and contractile dysfunction. Furthermore, IL-1 receptor blockade with anakinra is characterized by an extremely rapid onset of action and remarkable safety and may thus be suitable for the treatment of patients critically ill with myocarditis.

Highlights

  • A 65-year-old Caucasian man was hospitalized for low-grade fever, fatigue, and unintentional weight loss

  • Recent studies indicate that the pro-inflammatory cytokine interleukin (IL)-1 is central to the development of cardiac inflammation in the pathogenesis of a broad spectrum of conditions

  • The present study reports the dramatic efficacy of interleukin 1 (IL-1) blockade in a case of fulminant myocarditis

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Summary

INTRODUCTION

A 65-year-old Caucasian man was hospitalized for low-grade fever, fatigue, and unintentional weight loss He had a history of elevated arterial blood pressure and benign prostatic hyperplasia; family and social history were unremarkable. Blood tests showed mild leukocytosis (15,000/mm, neutrophils 88%), anemia (Hb 9.5 g/dL), thrombocytopenia (70,000/ mm3), high levels of inflammatory markers C-reactive protein (CRP, 104 mg/L, normal values 0–6 mg/L) and ferritin (515 ng/ mL, normal values 15–150 ng/mL), and mildly elevated liver transaminases and serum lactate dehydrogenase. Histologic examination was eventually diagnostic for anaplastic T cell lymphoma; macrophages phagocytizing hematopoietic cells were concomitantly observed. After this procedure, the patient acutely developed fever, hypotension, and dyspnea. During the following day, no signs of myocardial recovery were observed, and the severe biventricular dysfunction further worsened (LVEF < 20%)

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