Unexplained Catastrophic Systemic Neutrophilic Syndrome in a 68-Year-Old Man: A Severe Variant of Aseptic Abscesses or a New Entity?

Abstract

We report for the first time the case of a 68-year-old man with unexplained catastrophic systemic neutrophilic syndrome without abscesses. Although this case shares some features with autoinflammatory disorders, such as aseptic abscesses syndrome, including recurrent fever, leukocytosis, anemia, and elevated acute phase proteins, it is unique for the absence of deep abscess-like collections, the presence of high procalcitonin levels without infection, and a rapidly progressive, ultimately fatal condition despite aggressive therapies with high-dose corticosteroids and interleukin 1 receptor blockade with anakinra. We believe that this case presents unique characteristic features among autoinflammatory diseases. Further studies are needed to fully recognize this distinct clinical entity and clarify the role of neutrophil-derived cytokines in the pathogenic mechanisms of this life-threatening condition for the development of new treatment strategies. doi: http://dx.doi.org/10.4021/jmc1347w