Kawasaki disease shock syndrome: Unique and severe subtype of Kawasaki disease

Abstract

The presence of coronary aneurysms was significantly and positively correlated with male gender, IVIG resistance, inotrope treatment, cardiac failure, abdominal pain and neurological symptoms. IVIG-resistant patients had higher neutrophil : lymphocyte ratio. Abdominal symptoms, hypoalbuminemia and elevated C-reactive protein were present in almost all of the patients. Multisystem involvement with atypical presentation in KDSS is frequent. An important differential diagnosis is TSS. Mechanical ventilation, gastrointestinal and neurological symptoms were associated with IVIG resistance and the presence of coronary aneurysms. The first line of treatment includes IVIG and pulse corticosteroids; in severe cases, infliximab, anakinra, cyclosporine or plasmapheresis are alternative treatment options.

Anakinra Therapy for Non-cancer Inflammatory Diseases

“…Of note, the beneficial anti-inflammatory and therapeutic effects of IVIG in several immune-mediated disorders include a reduction in IL-1 production with an increase in IL-1Ra ( Aukrust et al, 1994 ). Consistently, reports on the efficacy of anakinra treatment indicate that IL-1 plays a pivotal role in the development of vascular damage in KD ( Cohen et al, 2012 ; Dusser and Kone-Paut, 2017 ; Blonz et al, 2018 ; Gamez-Gonzalez et al, 2018 ; Kone-Paut et al, 2018 ). A prospective trial on anakinra in KD is underway (NCT02179853).…”

A 13-Year-Old Boy Who Has Kawasaki Disease Shock Syndrome Presents with Parotitis

“…1 The recent description of KDSS shows a positive association between more severe laboratory markers of inflammation and a higher risk of coronary artery abnormalities, mitral regurgitation, inadequate response to IVIG, and prolonged myocardial dysfunction. 5,6 KDSS is distinguished from KD by most overt systolic and diastolic dysfunction and shock, thrombocytopenia, more severe laboratory markers of inflammation, and having an inadequate response to IVIG and a higher rate of coronary artery dilation and aneurysm formation. Although the overt systolic dysfunction resolved with vasoactive infusions in our patient, the abnormal ventricular diastolic function persisted into the chronic phase of the illness.…”

“…Although the overt systolic dysfunction resolved with vasoactive infusions in our patient, the abnormal ventricular diastolic function persisted into the chronic phase of the illness. [5][6][7] TSS is an exotoxin-mediated acute life-threatening illness which is usually caused by superantigens which are being produced by either S. aureus or group A Streptococcus. TSS is characterized by high-grade fever, rash, hypotension, multiorgan failure (involving at least three or more organ systems), and desquamation, typically of palms and soles.…”

“…KDSS is associated with more severe laboratory markers of inflammation and a higher risk of coronary artery abnormalities, mitral regurgitation, prolonged myocardial dysfunction, and resistance to immunoglobulin therapy. 5 We report a patient who presented with features of mumps and TSS. Later, the patient was diagnosed with KDSS when coronary artery aneurysms, mitral regurgitation, and prolonged myocardial dysfunction were detected (subsequently) on an echocardiographic examination.…”

Kawasaki disease shock syndrome: Case report and cytokine profiling

“…Since the first case of KDSS was reported, there appears to be an increased incidence of IVIG resistance and coronary abnormalities in patients with KDSS . Compared with KD patients without shock, KDSS tends to occur in older children, and is associated with pulmonary complications, AKI, pancreatitis, neurological disorders, coagulopathy, and hemophagocytosis.…”