Kawasaki Disease: an Unexpected Etiology of Shock and Multiple Organ Dysfunction Syndrome

Objective: Severe forms of Kawasaki disease (KD) associated with shock have recently been reported in which a greater number of coronary artery abnormalities (CAA) were observed. In this study, we analyzed organ involvement not restricted to cardiovascular aspects in severe KD and assessed whether their outcome is different than in common forms.

Design: Retrospective study.

Setting: A 12-bed pediatric intensive care unit (PICU) in a university hospital setting.

Patients: All patients managed in the PICU with a diagnosis of KD from 1 January 2001 to 30 April 2009.

Results: Eleven patients were admitted because of moderate febrile shock without initial KD diagnosis. Median age was 75 months (6-175) with a male:female ratio of 1.4. KD was diagnosed and treated after a delay of 1 day (0-2), for a total of 7 days (5-9) after fever onset. Seven patients (63%) developed CAA after 21 days (6-30) with complete regression within a delay of 120 days (18-240). Nonspecific encephalopathy (n = 6) as well as acute kidney injury (n = 10) were also observed. Multiple organ dysfunction syndrome (MODS) occurred in eight patients. Although predicted mortality according to the PELOD score [21 (10-43)] ranged from 20% to up to 50%, all 11 children survived with no sequelae.

Conclusion: Moderate shock is the main reason for PICU admission in children suffering from KD. These forms can be associated with surprising MODS. Despite the severity of symptoms, all patients survived without any sequelae, hence the need for proper diagnosis and rapid treatment of these unusual severe forms.