title: The hyperferritinemic syndrome: macrophage activation syndrome, Still's disease, septic shock and catastrophic antiphospholipid syndrome.
abstract: BACKGROUND
  Over the last few years, accumulating data have implicated a role for ferritin as a signaling molecule and direct mediator of the immune system. Hyperferritinemia is associated with a multitude of clinical conditions and with worse prognosis in critically ill patients.
  
  
  CONCLUSIONS
  There are four uncommon medical conditions characterized by high levels of ferritin, namely the macrophage activation syndrome (MAS), adult onset Still's disease (AOSD), catastrophic antiphospholipid syndrome (cAPS) and septic shock, that share a similar clinical and laboratory features, and also respond to similar treatments, suggesting a common pathogenic mechanism. Ferritin is known to be a pro-inflammatory mediator inducing expression of pro-inflammatory molecules, yet it has opposing actions as a pro-inflammatory and as an immunosuppressant. We propose that the exceptionally high ferritin levels observed in these uncommon clinical conditions are not just the product of the inflammation but rather may contribute to the development of a cytokine storm.
  
  
  CONCLUSIONS
  Here we review and compare four clinical conditions and the role of ferritin as an immunomodulator. We would like to propose including these four conditions under a common syndrome entity termed "Hyperferritinemic Syndrome".
authors: Center for Autoimmune Diseases, Sheba Medical Center affiliated with the Tel-Aviv University, Tel-Hashomer 52621, Israel.
authors: Cristina
authors: C
authors: Rosário
authors: Gisele
authors: G
authors: Zandman-Goddard
authors: Esther G
authors: EG
authors: Meyron-Holtz
authors: David P
authors: DP
authors: D'Cruz
authors: Yehuda
authors: Y
authors: Shoenfeld
mesh_terms: D000818:Animals; D016736:Antiphospholipid Syndrome / Q000097:blood* / Q000175:diagnosis / Q000453:epidemiology; D002386:Cataract / Q000097:blood / Q000151:congenital / Q000175:diagnosis / Q000453:epidemiology; D002388:Catastrophic Illness / Q000453:epidemiology; D005293:Ferritins / Q000097:blood*; D006801:Humans; D018836:Inflammation Mediators / Q000097:blood; D019189:Iron Metabolism Disorders / Q000097:blood / Q000151:congenital / Q000175:diagnosis / Q000453:epidemiology; D055501:Macrophage Activation Syndrome / Q000097:blood* / Q000175:diagnosis / Q000453:epidemiology; D012772:Shock, Septic / Q000097:blood* / Q000175:diagnosis / Q000453:epidemiology; D016706:Still's Disease, Adult-Onset / Q000097:blood* / Q000175:diagnosis / Q000453:epidemiology
chemical_list: D018836:Inflammation Mediators; D005293:Ferritins
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