We read the very timely article by Bhat,
et al. [1] providing valuable insights into clinical
epidemiology of multisystem inflammatory syndrome in
children (MIS-C). We comment on the recent evidence to
complement the information provided.
Recent clinical guidelines by American
College of Rheumatology (ACR) elaborate on the most
appropriate diagnostic and therapeutic steps for MIS-C at
the present time, advising inflammatory markers and cytokine
panel testing [2]. There is noteworthy discordance in
interleukin levels of IL-1, IL-6 and IL-10 among patients
with Kawasaki disease (KD) vs MIS-C [3]. While IL-1
is the main mediator of coronary artery inflammation in KD,
inflammatory process in MIS-C is predominantly driven by
IL-6 and IL-10, which may play a role in the myocardial
dysfunction and higher severity of the 2019-nCoV infection
[4].
We concur with the authors that the role
for specific cytokine blockade including use of biologics in
MIS-C is still lacking. The ACR guidelines advice
immunomodulatory therapy for all severe/critical MIS-C
patients with shock, significant respiratory distress,
neurologic changes, dehydration, or features of KD. IVIG and
glucocorticoid remain first line agents either alone or in
combination. Anakinra is safe in severe infections among
children with hyper-inflammatory syndromes. Although
tocilizumab is effective in reducing mortality and ICU
admission in patients with severe COVID-19 pneumonia [2],
the clinical evidence is insufficient regarding its efficacy
and safety for COVID-19 because of concerns regarding risk
of secondary bacterial and fungal infections [5]. Aspirin
(3-5 mg/kg/day) should be used in patients with MIS-C and
KD-like features and/or thrombocytosis and continued until
normali-zation of platelet count and confirmed normal
coronary arteries at ³4
weeks after diagnosis. Anticoagulation with enoxaparin
should be added in patients with coronary artery aneurysm
and Z score ³10.0
or an ejection fraction (EF) <35% [2], but despite benefits,
strategy based evidence is required due to high risk of
hemorrhagic events or complications.
With the availability of these guidelines
a standardized treatment plan for MIS-C involving
multidisciplinary care under pediatric cardiology,
infectious disease, intensive care and rheumatology
specialists can be designed. As the evidence base for
COVID-19 and MIS-C treatment and care management is evolving
rapidly, this guidance may change in future.
REFERENCES
1. Bhat CS, Gupta L, Balasubramanian S,
Singh S, Ramanan A V. Hyper inflammatory syndrome in
children associated with COVID-19: Need for awareness
[published online ahead of print, 2020 Jul 15]. Indian
Pediatr. 2020; S097475591600208.
2. Henderson LA, Canna SW, Friedman KG,
et al. American College of Rheumatology Clinical
Guidance for Pediatric Patients with Multisystem
Inflammatory Syndrome in Children (MIS-C) Associated with
SARS-CoV-2 and Hyper Inflam-mation in COVID-19. Version 1
[published online ahead of print, 2020 Jul 23]. Arthritis
Rheumatol. 2020;10.1002/art.41454.
3. Li H, Chen K, Liu M, Xu H, Xu Q. The
profile of peripheral blood lymphocyte subsets and serum
cytokines in children with 2019 novel coronavirus pneumonia.
J Infect. 2020; 81:115-20.
4. Shulman ST. Pediatric coronavirus
disease-2019-associated multisystem inflammatory syndrome. J
Pediatric Infect Dis Soc. 2020;9:285-6.
5. Cortegiani A, Ippolito M, Greco M, et al.
Rationale and evidence on the use of tocilizumab in
COVID-19: A systematic review. Pulmonol.
2020;S2531-0437:30153-7.
