Rare genetic variants involved in multisystem inflammatory syndrome in children: a multicenter Brazilian cohort study

Front Cell Infect Microbiol. 2023 Jul 31:13:1182257. doi: 10.3389/fcimb.2023.1182257. eCollection 2023.

Abstract

Introduction: Despite the existing data on the Multisystem Inflammatory Syndrome in Children (MIS-C), the factors that determine these patients evolution remain elusive. Answers may lie, at least in part, in genetics. It is currently under investigation that MIS-C patients may have an underlying innate error of immunity (IEI), whether of monogenic, digenic, or even oligogenic origin.

Methods: To further investigate this hypothesis, 30 patients with MIS-C were submitted to whole exome sequencing.

Results: Analyses of genes associated with MIS-C, MIS-A, severe covid-19, and Kawasaki disease identified twenty-nine patients with rare potentially damaging variants (50 variants were identified in 38 different genes), including those previously described in IFNA21 and IFIH1 genes, new variants in genes previously described in MIS-C patients (KMT2D, CFB, and PRF1), and variants in genes newly associated to MIS-C such as APOL1, TNFRSF13B, and G6PD. In addition, gene ontology enrichment pointed to the involvement of thirteen major pathways, including complement system, hematopoiesis, immune system development, and type II interferon signaling, that were not yet reported in MIS-C.

Discussion: These data strongly indicate that different gene families may favor MIS- C development. Larger cohort studies with healthy controls and other omics approaches, such as proteomics and RNAseq, will be precious to better understanding the disease dynamics.

Keywords: Kawasaki disease; coronavirus infection; mucocutaneous lymph node syndrome; multisystem inflammatory syndrome in children; pediatric inflammatory multisystem syndrome; whole exome sequencing.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Apolipoprotein L1
  • Brazil
  • COVID-19* / complications
  • COVID-19* / genetics
  • Child
  • Cohort Studies
  • Humans
  • Systemic Inflammatory Response Syndrome

Substances

  • APOL1 protein, human
  • Apolipoprotein L1

Supplementary concepts

  • adult multisystem inflammatory disease, COVID-19 related
  • pediatric multisystem inflammatory disease, COVID-19 related

Grants and funding

This study was supported by grants from the following Brazilian research promotion agencies: Inova Fiocruz/Fundação Oswaldo Cruz and Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq) (grant 308697/2019-7 and 310299/2022-5) to FP. Conselho Nacional de Desenvolvimento Científico e Tecnológico – CNPq (National Council for Scientific and Technological Development – CNPq), grant n° 309482/2022-4 to O-CM and 401597/2020-2, Fundação Carlos Chagas Filho de Amparo à Pesquisa do Estado do Rio de Janeiro – FAPERJ (Carlos Chagas Filho Foundation for Research Support of the State of Rio de Janeiro – FAPERJ), Process n° E-26/010.000160/2020, grant n° 2020/0996, Fundação de Amparo à Pesquisa do Estado de São Paulo – FAPESP (São Paulo Research Foundation – FAPESP) grants 2018/18886-9, 2020/01688-0, and 2020/07069-0 to O-CM, 2020/16246-2 to DLMF, and 2020/11710-2 to DP, Post-Doctoral Fellowship from FAPERJ (E-26/203.910/2021) to RSF, The APC was funded by CAPES (Finance Code 001) through our institutional Post-Graduate Program “Pesquisa Aplicada à Sauíde da Criança e da Mulher”.