Kawasaki Disease Shock Syndrome: A Challenging Diagnosis
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Kawasaki disease (KD) is the most common pediatric cause of acquired heart disease in developmental countries and can lead to permanent changes in the coronary arteries in up to 25% of untreated cases. However, initial presentation with hemodynamic instability is not common. Kawasaki disease shock syndrome (KDSS) was first described in 2009. The cause of severe Kawasaki disease shock syndrome hypotension is not yet fully known, although it is believed to be due to vasculitis along with capillary leakage, myocardial dysfunction, and generalized cytokine dysregulation. Kawasaki disease shock syndrome is an under-diagnosed entity, with an estimated occurrence in 5-7% of children with Kawasaki disease.

A previously healthy 4-year-old girl, admitted to a pediatric intensive care unit (PICU) presenting with a fluid-refractory shock. She was initially admitted in the pediatric emergency department with a history of fever and petechial rash and an initial diagnosis of invasive meningococcal disease was placed. During the PICU stay, acute myocarditis with episodes of supraventricular tachycardia and lateral and inferior wall myocardial ischemia, ileitis, and serositis were noted. On the 14th day of stay, she developed peeling on the hands and feet and, aneurysmatic ectasia of the coronary arteries was visualized in transthoracic echocardiography, allowing the diagnosis of Kawasaki disease shock syndrome. On day 1 of admission to the PICU, empiric antimicrobial therapy with vancomycin and metronidazole was started, both to cover for Gram-positive and anaerobic bacteria. Acyclovir and ceftriaxone were suspended on day 2. Due to an unexplained prolonged fever, raised inflammatory parameters and increased abdominal distension, empiric treatment with meropenem was initiated to cover for a potential intraabdominal infection. She completed a total of 19 days of vancomycin (20 mg/kg IV q12h), 16 days of ciprofloxacin (10 mg/kg IV q12h), 15 days of meropenem (20 mg/kg IV q12h), and 7 days of metronidazole (7.5 mg/kg IV q8h). She was put on methylprednisolone (1 mg/kg/day) on day 4, due to the suspicion of the hemophagocytic syndrome, which was changed on day 17 to prednisolone (1 mg/kg PO q12h), until day 24, after gradual reduction.

Several blood analyses were performed, showing anemia (minimum Hb 6.8 g/dL) and thrombocytopenia (minimum of 12,000/µL platelets). The minimum fibrinogen assay was 87 g/dL, and the maximum ferritin was 5,070 ng/mL. Furthermore, on suspicion of secondary macrophage activation syndrome (prolonged fever, rash, hypofibrinogenemia, hyperferritinemia, and bicytopenia), a medullogram was performed on day 5, which could suggest reactive neutropoiesis, without hemophagocytosis. A transfusion of red blood cells was given on day 4 for anemia and platelet transfusion for thrombocytopenia on day 5, day 8, and day 9. The patient was transferred to her local hospital 25 days after admission to the PICU, where she was hospitalized for another 18 days. Another echocardiogram was performed before discharge with similar findings and a 12-lead ECG was normal. She underwent a program of rehabilitation due to reduced muscle strength in the lower limbs and made a good recovery. She maintained prednisolone, acetylsalicylic acid, and clopidogrel as home therapy with gradual reduction.

She is currently being followed up in the cardiology and rheumatology consultation. Nine months after being discharged, she is asymptomatic. In the last echocardiogram, no ectasias or other alterations were visible and only therapy with acetylsalicylic acid is maintained.