Ebola Virus Disease has Features of Hemophagocytic Lymphohistiocytosis Syndrome

The 2014 epidemic of Ebola virus disease (EVD) in several West African countries has mortality rates of up to 70%, as reported by WHO. The exponential increase in the number of infections and deaths, coupled with the absence of specific preventive and therapeutic strategies, represents one of the biggest global health challenges of this millenium. Insights in the pathophysiology and treatment of EVD are thus urgently needed. In this paper, we postulate that EVD has the characteristics of hemophagocytic lymphohistiocytosis (HLH) syndrome (syn., macrophage activation syndrome). The identification of HLH as part of severe EVD, however, brings the possibility for a pathophysiologically targeted approach of therapy; special promise is represented by cytokine-directed therapy in the form of recombinant interleukin-1 receptor antagonist (anakinra). Treatment with anakinra has the advantage to interrupt the deleterious IL-1-mediated hyperinflammatory loop in macrophage activation syndrome, and it has been shown to be associated with remarkable effectiveness and an excellent safety record.