Kawasaki shock syndrome complicating a recurrence of Kawasaki disease

Pediatrics. 2014 Dec;134(6):e1695-9. doi: 10.1542/peds.2014-0004. Epub 2014 Nov 10.

Abstract

We describe a case of recurrent Kawasaki disease (KD) in a non-Asian 6-year-old boy who had been diagnosed with typical KD without cardiac involvement at age 3 years. He was admitted to the PICU 3 years later for heart failure, hypotension, and deterioration of his general condition. Ultrasonography revealed left ventricular dysfunction with a 44% ejection fraction and grade I mitral valve failure without coronary artery involvement. Subsequent observation of hyperemic conjunctiva, bilateral cervical adenopathies with erythematous skin (normal neck ultrasound and computed axial tomography findings), peeling of the fingertips at day 8 of the illness, and occurrence of an inflammatory syndrome led to a diagnosis of incomplete recurrent KD with a clinical picture of Kawasaki shock syndrome (KSS). Clinical improvement was rapidly obtained after intravenous immunoglobulin and intravenous corticosteroid therapy (30 mg/kg per day for 3 subsequent days). Left ventricular function gradually improved, with ultrasound returning to normal after 3 months. Diagnosis was difficult to establish because of the recurrence of the disease and the incomplete clinical picture, with clinical features of KSS. Physicians need to be aware of these pitfalls in the management of patients with clinical signs of KD.

Keywords: case report; drug therapy/combination; glucocorticoids/therapeutic use; mucocutaneous lymph node syndrome; mucocutaneous lymph node syndrome/diagnosis; mucocutaneous lymph node syndrome/therapy; recurrence; shock/diagnosis.

Publication types

  • Case Reports

MeSH terms

  • Aspirin / administration & dosage
  • Child
  • Combined Modality Therapy
  • Diagnosis, Differential
  • Dose-Response Relationship, Drug
  • Drug Administration Schedule
  • Echocardiography
  • Heart Failure / diagnosis*
  • Heart Failure / therapy
  • Humans
  • Hypotension / diagnosis*
  • Hypotension / therapy
  • Immunization, Passive
  • Inflammation Mediators / blood
  • Intensive Care Units, Pediatric
  • Male
  • Methylprednisolone / administration & dosage
  • Mucocutaneous Lymph Node Syndrome / diagnosis*
  • Mucocutaneous Lymph Node Syndrome / therapy
  • Recurrence
  • Shock / diagnosis*
  • Shock / therapy

Substances

  • Inflammation Mediators
  • Aspirin
  • Methylprednisolone