OBJECTIVE. We sought to define the characteristics that distinguish Kawasaki disease shock syndrome from hemodynamically normal Kawasaki disease.
METHODS. We collected data prospectively for all patients with Kawasaki disease who were treated at a single institution during a 4-year period. We defined Kawasaki disease shock syndrome on the basis of systolic hypotension for age, a sustained decrease in systolic blood pressure from baseline of ≥20%, or clinical signs of poor perfusion. We compared clinical and laboratory features, coronary artery measurements, and responses to therapy and analyzed indices of ventricular systolic and diastolic function during acute and convalescent Kawasaki disease.
RESULTS. Of 187 consecutive patients with Kawasaki disease, 13 (7%) met the definition for Kawasaki disease shock syndrome. All received fluid resuscitation, and 7 (54%) required vasoactive infusions. Compared with patients without shock, patients with Kawasaki disease shock syndrome were more often female and had larger proportions of bands, higher C-reactive protein concentrations, and lower hemoglobin concentrations and platelet counts. Evidence of consumptive coagulopathy was common in the Kawasaki disease shock syndrome group. Patients with Kawasaki disease shock syndrome more often had impaired left ventricular systolic function (ejection fraction of <54%: 4 of 13 patients [31%] vs 2 of 86 patients [4%]), mitral regurgitation (5 of 13 patients [39%] vs 2 of 83 patients [2%]), coronary artery abnormalities (8 of 13 patients [62%] vs 20 of 86 patients [23%]), and intravenous immunoglobulin resistance (6 of 13 patients [46%] vs 32 of 174 patients [18%]). Impairment of ventricular relaxation and compliance persisted among patients with Kawasaki disease shock syndrome after the resolution of other hemodynamic disturbances.
CONCLUSIONS. Kawasaki disease shock syndrome is associated with more-severe laboratory markers of inflammation and greater risk of coronary artery abnormalities, mitral regurgitation, and prolonged myocardial dysfunction. These patients may be resistant to immunoglobulin therapy and require additional antiinflammatory treatment.
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.
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Conflict of Interest:
None declared