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ARTICLES: John T. Kanegaye, Matthew S. Wilder, Delaram Molkara, Jeffrey R. Frazer, Joan Pancheri, Adriana H. Tremoulet, Virginia E. Watson, Brookie M. Best, and Jane C. Burns Recognition of a Kawasaki Disease Shock Syndrome Pediatrics 2009; 123: e783-e789 [Abstract] [Full text] [PDF]

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Shock syndrome, or macrophage activation syndrome, in children with Kawasaki disease

Randy Q Cron   (13 May 2009)

Shock syndrome, or macrophage activation syndrome, in children with Kawasaki disease 13 May 2009
Randy Q Cron, physician Children's Hospital of Alabama Send letter to journal: Re: Shock syndrome, or macrophage activation syndrome, in children with Kawasaki disease rcron{at}peds.uab.edu Randy Q Cron	To the Editor: I read with interest Kanegaye and colleagues’ recent report regarding shock syndrome (SS) in children with Kawasaki disease (KD).(1) The authors report on 13 children with KD and SS and compare them to 174 KD children without SS. SS was defined as sustained hypotension or signs of poor perfusion. In addition to hemodynamic instability, the SS group had significantly lower age-adjusted hemoglobin z scores and higher C reactive protein levels. There were also strong trends for the SS patients to have lower erythrocyte sedimentation rates (ESR) and white blood cell counts, along with higher liver function tests. Interestingly, the SS group had lower platelet counts, positive D-dimers, and elevated partial thromboplastin times, all consistent with coagulopathy. All of these afore noted features are characteristic for macrophage activation syndrome (MAS) or hemophagocytic lymphohistiocytosis.(2) MAS is the result of a cytokine storm and associated hemophagocytosis that results in a consumptive coagulopathy that is often fatal.(3) MAS is a relatively frequent complication of rheumatic diseases, and there have been several cases reported among children with KD.(4-10) Typically, during MAS the ESR begins to fall, as do all 3 major cell lines in the blood.(2) Concurrently, a sepsis-like picture develops with evidence of hemophagocytosis, coagulopathy, and liver dysfunction.(2) A hallmark of the disorder is inordinately elevated ferritin levels, typically several thousand ng/ml.(11) It would be of interest to know the ferritin values in the KD patients with and without SS reported by Kanegaye and colleagues(1) to better assess the likelihood of MAS.(11) Treatment of MAS usually consists of high dose corticosteroids and cyclosporin.(12) Recently, biologic modifiers such as IL-1 antagonists have proven useful in the treatment of refractory MAS,(13, 14) including a patient with KD.(6) Thus, the diagnosis of MAS in children with KD is critical for optimal therapy and prevention of poor outcomes. REFERENCES 1. Kanegaye JT, Wilder MS, Molkara D, Frazer JR, Pancheri J, Tremoulet AH, et al. Recognition of a Kawasaki disease shock syndrome. Pediatrics 2009;123(5):e783-9. 2. Grom AA. Macrophage activation syndrome and reactive hemophagocytic lymphohistiocytosis: the same entities? Curr Opin Rheumatol 2003;15(5):587 -90. 3. Behrens EM. Macrophage activation syndrome in rheumatic disease: what is the role of the antigen presenting cell? Autoimmun Rev 2008;7(4):305-8. 4. al-Eid W, al-Jefri A, Bahabri S, al-Mayouf S. Hemophagocytosis complicating Kawasaki disease. Pediatr Hematol Oncol 2000;17(4):323-9. 5. Avcin T, Tse SM, Schneider R, Ngan B, Silverman ED. Macrophage activation syndrome as the presenting manifestation of rheumatic diseases in childhood. J Pediatr 2006;148(5):683-6. 6. Cron RQ, Beukelman T, Reed AB, Behrens EM. IL-1 antagonism for treatment of macrophage activation syndrome (abstract). Arthritis Rheum 2008;58 (suppl 9):S247-8. 7. Cummings C, McCarthy P, van Hoff J, Porter G, Jr. Kawasaki disease associated with reactive hemophagocytic lymphohistiocytosis. Pediatr Infect Dis J 2008;27(12):1116-8. 8. Muise A, Tallett SE, Silverman ED. Are children with Kawasaki disease and prolonged fever at risk for macrophage activation syndrome? Pediatrics 2003;112(6 Pt 1):e495. 9. Palazzi DL, McClain KL, Kaplan SL. Hemophagocytic syndrome after Kawasaki disease. Pediatr Infect Dis J 2003;22(7):663-6. 10. Thabet F, Bellara I, Tabarki B, Kchaou H, Selmi H, Yacoub M, et al. [Ischemic colitis and hemophagocytosis complicating Kawasaki disease]. Arch Pediatr 2004;11(3):226-8. 11. Ravelli A. Macrophage activation syndrome. Curr Opin Rheumatol 2002;14(5):548-52. 12. Kelly A, Ramanan AV. Recognition and management of macrophage activation syndrome in juvenile arthritis. Curr Opin Rheumatol 2007;19(5):477-81. 13. Behrens EM, Kreiger PA, Cherian S, Cron RQ. Interleukin 1 receptor antagonist to treat cytophagic histiocytic panniculitis with secondary hemophagocytic lymphohistiocytosis. J Rheumatol 2006;33(10):2081-4. 14. Kelly A, Ramanan AV. A case of macrophage activation syndrome successfully treated with anakinra. Nat Clin Pract Rheumatol 2008;4(11):615-20. Conflict of Interest: None declared