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

Kawasaki disease shock syndrome: A report of two cases and literature review

“…Previous patients had significantly increased WBC, low platelet counts, and low hemoglobin and serum albumin levels but high CRP levels. These patients also tended to not respond to IVIG, and also had higher incidence of coronary artery dilatation or evolving aneurysms . Both of our patients met these characteristics.…”

“…Lin, et al stated that, echocardiographic abnormities, especially coronary artery lesions, anemia, and thrombocytosis were highly suggestive of KDSS . However, in most studies, including in our two patients, in acute KDSS, lower platelet counts were more common . Given these considerations, it is very important to make a correct diagnosis using echocardiography to detect coronary artery lesions and to give appropriate treatment .…”

Clinical features of acute kidney injury in patients with Kawasaki disease

“…KDSS: KDSS is a condition characterized by systolic hypotension or clinical signs of poor perfusion in patients with KD[49,50]. Although the pathogenesis underlying KDSS is unknown, intensive vasculitis with capillary leak and hypercytokinemia with myocardial dysfunction may cause KDSS[49-51].…”

Kawasaki disease: An ongoing challenge

“…7,9,12 Risk factors in developing KDSS include female gender, higher CRP levels, anaemia, thrombocytopenia and deranged LFTs, 7 all of which were present in our case. This uncommon complication is known as 'Kawasaki disease shock syndrome' (KDSS).…”