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Clinical Rheumatology

, Volume 36, Issue 1, pp 223–228 | Cite as

Describing Kawasaki shock syndrome: results from a retrospective study and literature review

  • Andrea TaddioEmail author
  • Eleonora Dei Rossi
  • Lorenzo Monasta
  • Serena Pastore
  • Alberto Tommasini
  • Loredana Lepore
  • Gabriele Bronzetti
  • Edoardo Marrani
  • Biancamaria D’Agata Mottolese
  • Gabriele Simonini
  • Rolando Cimaz
  • Alessandro Ventura
Case Based Review

Abstract

Kawasaki shock syndrome (KSS) is a rare manifestation of Kawasaki disease (KD) characterized by systolic hypotension or clinical signs of poor perfusion. The objectives of the study are to describe the main clinical presentation, echocardiographic, and laboratory findings, as well as the treatment options and clinical outcomes of KSS patients when compared with KD patients. This is a retrospective study. All children referred to two pediatric rheumatology units from January 1, 2012, to December 31, 2014, were enrolled. Patients were divided into patients with or without KSS. We compared the two groups according to the following variables: sex, age, type of KD (classic, with less frequent manifestations, or incomplete), clinical manifestations, cardiac involvement, laboratory findings, therapy administered, response to treatment, and outcome. Eighty-four patients with KD were enrolled. Of these, five (6 %) met the criteria for KSS. Patients with KSS had higher values of C-reactive protein (p = 0.005), lower hemoglobin levels (p = 0.003); more frequent hyponatremia (p = 0.004), hypoalbuminemia (p = 0.004), and coagulopathy (p = 0.003); and increase in cardiac troponins (p = 0.000). Among the KSS patients, three had a coronary artery involvement, but none developed a permanent aneurysm. Intravenous immunoglobulin resistance was more frequent in the KSS group, although not significantly so (3/5, 60 % vs. 23/79, 30 %, P = NS). None of the five cases was fatal, and all recovered without sequelae. KSS patients are more likely to have higher rates of cardiac involvement. However, most cardiovascular abnormalities resolved promptly with therapy.

Keywords

Coronary artery involvement Heart failure Hypotension Kawasaki disease Kawasaki shock syndrome 

Notes

Acknowledgments

The authors do not have any funding to declare

Compliance with ethical standards

Disclosures

None.

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Copyright information

© International League of Associations for Rheumatology (ILAR) 2016

Authors and Affiliations

  • Andrea Taddio
    • 1
    • 2
    Email author
  • Eleonora Dei Rossi
    • 1
  • Lorenzo Monasta
    • 2
  • Serena Pastore
    • 2
  • Alberto Tommasini
    • 2
  • Loredana Lepore
    • 1
  • Gabriele Bronzetti
    • 3
  • Edoardo Marrani
    • 4
  • Biancamaria D’Agata Mottolese
    • 2
  • Gabriele Simonini
    • 4
  • Rolando Cimaz
    • 4
  • Alessandro Ventura
    • 1
    • 2
  1. 1.University of Study of TriesteTriesteItaly
  2. 2.Institute for Maternal and Child Health—IRCCS “Burlo Garofolo”TriesteItaly
  3. 3.Cardiologia Pediatrica e dell’Età Evolutiva, Policlinico Sant’Orsola—MalpighiBolognaItaly
  4. 4.AOU MeyerFlorenceItaly

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