P KD SHOCK SYNDROME

Twelve patients diagnosed with KDSS were retrospectively reviewed from two centers in Mexico, along with 91 additional cases from the literature. Sixty patients presented with complete KD (68%), and 45.6% (45/103) of the patients had unusual KD manifestations. The most frequent diagnosis at the time of admission was toxic shock syndrome (TSS) (20 patients). Sixteen of them presented coronary artery abnormalities. Overall, abnormalities in the coronary arteries were documented in 72.8% of the patients. The mortality rate was 6.8%. The presence of coronary aneurysms showed a statistically significant positive correlation with male gender, IVIG‐resistance, inotropic treatment, cardiac failure, abdominal pain and neurological manifestations. IVIG‐resistant patients showed higher values of neutrophil‐lymphocyte ratio (NLR). Abdominal manifestations, hypoalbuminemia and elevated CRP are constant findings. Multisystemic involvement with atypical presentation in KDSS is frequent. An important differential diagnosis is TSS. Mechanical ventilation, gastrointestinal and neurological manifestations were associated with IVIG‐resistance and the presence of coronary aneurysms. The first line of treatment includes IVIG and corticosteroids in pulses, and in severe cases infliximab, anakinra, cyclosporine or plasmapheresis are alternative treatment options.