Variation in Early Anakinra Use and Short-Term Outcomes in Multisystem Inflammatory Syndrome in Children.

OBJECTIVE: Evidence regarding effectiveness of interleukin-1 receptor antagonism in multisystem inflammatory syndrome in children (MIS-C) is lacking. We characterized variation in initial treatment with anakinra and evaluated cardiovascular outcomes associated with adding anakinra to standard initial therapy. METHODS: We conducted a retrospective cohort study of MIS-C cases in a US surveillance registry from November 2020 to December 2021. Day 0 was the first calendar day of immunomodulatory treatment. Factors associated with initial anakinra use (days 0-1) were identified. We compared cases in patients ages 2-20 years receiving intravenous immunoglobulin (IVIG) and glucocorticoids versus anakinra plus IVIG and/or glucocorticoids on days 0-1, using inverse probability weighting to balance disease severity. Primary outcomes were vasopressor requirement on day 3 and impaired left ventricular ejection fraction on days 3-4. The secondary outcome was 50% reduction in C-reactive protein on day 3. RESULTS: Among 1,516 MIS-C cases at 44 sites, 193 (13%) patients received anakinra alone or with other immunomodulators as initial treatment (range 0-74% by site). Site accounted for 59% of residual variance in anakinra use. After balancing disease severity, initial treatment with anakinra plus IVIG and/or glucocorticoids (n = 121) versus IVIG plus glucocorticoids (n = 389) was not associated with significant differences in vasopressor requirement (25.6% versus 20.1%, respectively; risk ratio [RR] 1.27 [95% confidence interval (95% CI) 0.88-1.84]), ventricular dysfunction (33.7% versus 25.7%, respectively; RR 1.31 [95% CI 0.98-1.75]), or C-reactive protein reduction. CONCLUSION: We identified substantial variation in initial anakinra use in a real-world population of children with MIS-C, but no average short-term improvement in cardiovascular outcomes associated with early addition of anakinra to IVIG and/or glucocorticoids compared to IVIG and glucocorticoids alone.

Comprehensive left ventricular myocardial deformation assessment in children with Kawasaki disease.

OBJECTIVE: Children with Kawasaki disease (KD) with persistent coronary artery aneurysms (CAAs) can develop chronic vasculopathy and subsequent myocardial ischemia. Early detection of this process is challenging. Myocardial deformation analysis can detect early alterations in myocardial performance. We aim to determine whether there are differences in myocardial deformation between KD patients with and without CAAs. DESIGN: This is a cross-sectional study of 123 echocardiograms performed on 103 children with KD. Myocardial deformation was measured with two-dimensional speckle tracking (2DSTE). The echocardiograms were divided into groups according to the KD phase in which they were performed: acute, subacute, and convalescent/chronic. The convalescent/chronic phase group was then divided based on the presence or absence of CAAs. Left ventricular (LV) global longitudinal strain (GLS), global longitudinal strain rate (GLSSR), global circumferential strain (GCS), global circumferential systolic strain rate (GCSSR), peak torsion, and torsion rate were measured. RESULTS: The numbers of echocardiograms analyzed in each of the KD phase groups were: 31 acute, 25 subacute, and 67 convalescent/chronic. Myocardial deformation was within normal limits in all groups. However, GLSSR, GCSSR, peak torsion, and torsion rate were lower in the convalescent/chronic phase group than in the acute phase group (mean, -1.37 ± 0.24 vs -1.55 ± 0.21 1/s; -1.63 ± 0.27 vs -1.84 ± 0.35 1/s; 2.49 ± 1.13 vs 3.41 ± 2.60 °/cm, and 21.97 ± 8.36 vs 26.69 ± 10.86 °/cm/s; P < .05 for all). The convalescent/chronic phase subgroup with CAAs had lower GLSSR and GCSSR than the subgroup without CAAs (mean, -1.23 ± 0.22 vs -1.42 ± 0.22 1/s; -1.46 ± 0.25 vs -1.68 ± 0.26 1/s, P < .05 for both). CONCLUSIONS: Children in the convalescent/chronic phase of KD have a subtle decrease in strain rate when compared to the acute phase, although within the normal range. This decrease is more pronounced in children with CAAs than those without CAAs. Longitudinal studies are needed to discern whether low-normal strain rate predicts decreased myocardial function in the long term.