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FRI0530 Kawasaki Disease Shock Syndrome – A Serious and Atypical Clinical Variant of Kawasaki Disease. Coimbra Series' from 1980 to 2014
  1. I.R. Luz1,
  2. L. Quaresma2,3,
  3. P. Estanqueiro2,3,
  4. L. Carvalho3,
  5. F. Neves3,
  6. M. Salgado2,3
  1. 1Paediatric Medical Service
  2. 2Reumatology Unit
  3. 3Paediatric Intensive Care Unit (PICU), Hospital Pediátrico, Coimbra, Coimbra, Portugal


Background Hemodynamic instability can occur in 7% of patients in the acute phase of Kawasaki disease (KD). This condition, denominated Kawasaki disease shock syndrome (KDSS), is characterized by myocardial dysfunction, shock, early coronary involvement, worse response to intravenous immunoglobulin (IVIG), more incomplete presentations and worse prognosis.

Objectives Determine the prevalence of KDSS in the past 34 years in our tertiary hospital.

Methods Retrospective study of PICU charts from children admitted from January 1980 to December 2014. KDSS was defined on the basis of clinical characteristics of KD and hypotension, shock or sepsis.

Results We identified 7 children (5 males), 3 without KD diagnosis in admission. 2 were under 12 months of age and 4 had ≥5 years. All had fever that lasted at least 5 days; conjunctivitis, changes of lips and oral cavity, changes in extremities and rash were present in 5 cases; cervical lymphadenopathy in 2; incomplete KD in 3.

In the acute phase all had leucocytosis with neutrophilia, 5 had thrombocytopenia, hypoalbuminemia and hyponatremia; 5 had congestive heart failure (CHF), 2 acute kidney injury (AKI) (1 needed dialysis), ascites and gallbladder hydrops.

Echocardiographic abnormalities: coronary 5 (aneurysm 2), pericardial effusion 4, myocarditis/mitral regurgitation 3, iliac arteries' aneurysm 1.

Treatments: intravenous fluid resuscitation 4, vasoactive agents 7, intravenous immunoglobulin therapy 7 (>1 course: 3), steroids 2.

Conclusions We found incomplete forms of KD in 43% of KDSS cases. There was a high prevalence of age >5 years old (57%), thrombocytopenia (71%), CHF (71%), AKI (29%), coronary abnormalities (71%) and IVIG resistance (43%). Paediatricians should be aware of this serious and atypical clinical variant of KD, many times incomplete, in order to make an earlier diagnosis and treatment and so improve prognosis.


  1. Kanegaye JTet al. Recognition of a Kawasaki disease shock syndrome. Pediatrics 2009;123:e783e9.

  2. Gámez-González LB et al. Clinical manifestations associated with Kawasaki disease shock syndrome in Mexican children. Eur J Pediatr 2013;172:337–342.

  3. Dominguez SR et al. Kawasaki Disease in a Pediatric Intensive Care Unit: A Case-Control Study. Pediatrics 2008;122:e786–e790.

Disclosure of Interest None declared

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