A new systemic inflammatory syndrome resembling Kawasaki disease (KD) may be temporally associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in children, according to a case series published in Annals of the Rheumatic Diseases. Data collected from France indicated that the Kawasaki-like disease associated with SARS-CoV-2 infection was distinct from typical KD observed in patients without the infection.

A retrospective analysis was conducted using data from 2 national referral networks in 7 different regions in Paris, France, since April 2020. Patients aged <18 years with confirmed SARS-CoV-2 infection or in close contact with an individual who tested positive for SARS-CoV-2 were eligible for inclusion in the study. Kawasaki disease was defined as persistent fever over 5 days with at least 4 of the characteristics including conjunctivitis, lymphadenopathy, skin rash, red and cracked lips, and inflammation of hands and feet. Severe disease was defined as the need for intensive care and/or a fatal outcome. Data from patients with Kawasaki-like disease and SARS-CoV-2 infection were compared with those from a historic cohort of children with KD alone. The control cohort was assembled from data collected at the same clinics between 2005 and 2020. Descriptive statistics were used to compare the historic KD cohort with the coronavirus disease 2019 (COVID-19)-associated Kawasaki-like disease or the “Kawa-COVID-19” cohort.

A total of 16 patients were included in analyses (50% boys; median age, 10 years at hospital admission). Eleven patients (69%) had laboratory-confirmed SARS-CoV-2 infection, and 5 reported recent contact with a laboratory-confirmed COVID-19 case. Median time from Kawa-COVID-19 symptoms onset to hospitalization was 5 days (IQR, 4-7 days). The main features of Kawa-COVID-19 included mucocutaneous involvement, conjunctivitis, dry cracked lips, cervical lymphadenopathy, and arthritis. Less typical features of KD were also observed, which could be linked to SARS-CoV-2 infection, including gastrointestinal symptoms, hemodynamic failure, neurologic symptoms, respiratory symptoms, Raynaud syndrome, and anosmia. A total of 5 patients (31%) were successfully treated with a single intravenous immunoglobin infusion; the remaining 10 required a second line of treatment. Severe disease occurred in 7 patients. Patients with severe vs nonsevere disease were older (median age, 12 vs 5 years; P =.043) and had higher ferritin levels (median, 1760 vs 295 μg/L; P =.003). All patients were in remission after a median of 2 days (IQR, 1-8 days).

Compared with the historic KD cohort, patients with Kawa-COVID-19 were significantly older (median age, 2 vs 10 years), had lower platelet and lymphocyte counts (383 vs 188 G/L and 3.08 vs 1.15 G/L, respectively; P <.0001 for both), higher frequency of cardiac involvement and pericarditis (1% vs 44% and 7% and 25%; P =.0001 and P =.029, respectively). In addition, patients in the Kawa-COVID-19 group vs the control group had greater resistance to first intravenous immunoglobulin treatment (62% vs 20%; P =.004).


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Overall, systemic inflammatory disease mimicking KD appeared to be a consequence of SARS-CoV-2 infection in some children, which may challenge the notion that COVID-19 is typically benign in children. According to the authors of this case series, appropriate management of this phenotype is rapid and aggressive and includes initial intravenous immunoglobin infusions and second-line steroids, if necessary.

“Further prospective international collections of Kawa-COVID-19, including the assessment of immunologic and host genetic factors, are necessary to better understand this novel disease entity,” they wrote.

Reference

Pouletty M, Borocco C, Ouldali N, et al. Paediatric multisystem inflammatory syndrome temporally associated with SARS-CoV-2 mimicking Kawasaki disease (Kawa-COVID-19): a multicentre cohort [published online June 11, 2020]. Ann Rheum Dis. doi:10.1136/annrheumdis-2020-217960