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Whole-cell MALDI-TOF Mass Spectrometry is an Accurate and Rapid Method to Analyze Different Modes of Macrophage Activation
Published on: December 26, 2013
Macrophage activation syndrome associated with multi-system inflammatory syndrome in children: a case report and
Yuji Fujita1, Masaki Shimizu2, Sayaka Aihara1
1Department of Pediatrics, Dokkyo Medical University, Tochigi, Japan.
Insights
Macrophage activation syndrome (MAS) can complicate multisystem inflammatory syndrome in children (MIS-C). Diagnosis and treatment for MIS-C/MAS may align with systemic juvenile idiopathic arthritis/MAS criteria, with elevated CXCL9 levels noted.
Area of Science:
- Pediatrics
- Rheumatology
- Infectious Diseases
- Immunology
Background:
- Macrophage activation syndrome (MAS) is a severe complication of rheumatic diseases like systemic juvenile idiopathic arthritis (sJIA).
- Multisystem inflammatory syndrome in children (MIS-C), linked to SARS-CoV-2, has been observed to present with MAS.
- Diagnostic criteria for MIS-C-associated MAS (MIS-C/MAS) are not yet established.
Purpose of the Study:
- To describe a case of MIS-C/MAS in a pediatric patient.
- To investigate the cytokine profile in MIS-C/MAS.
- To explore potential diagnostic and therapeutic parallels between MIS-C/MAS and sJIA/MAS.
Main Methods:
- Case report of a 12-year-old boy with MIS-C/MAS following COVID-19.
- Diagnosis based on adapted sJIA/MAS criteria.
- Treatment with intravenous immunoglobulin, glucocorticoids, and cyclosporine.
- Analysis of serum cytokine profile, including CXCL9 levels.
Main Results:
- The patient was successfully diagnosed and treated for MIS-C/MAS without coronary sequelae.
- Significantly elevated CXCL9 levels were observed, similar to sJIA/MAS.
- Cytokine profiles suggest potential consistency between MIS-C/MAS and sJIA/MAS diagnostic criteria.
Conclusions:
- MIS-C can be complicated by MAS, requiring consideration by pediatricians.
- Diagnostic criteria for sJIA/MAS may be applicable to MIS-C/MAS.
- Elevated CXCL9 levels are a potential biomarker for MIS-C/MAS.
- Glucocorticoids and cyclosporine are potential treatment options for MIS-C/MAS.
Abstract:
Macrophage activation syndrome (MAS) is a potentially life-threatening complication requiring early diagnosis and prompt treatment in rheumatic diseases such as systemic juvenile idiopathic arthritis (sJIA). Recently, multisystem inflammatory syndrome in children (MIS-C) associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection was reported to be complicated by MAS. The diagnostic criteria for MAS-associated MIS-C (MIS-C/MAS) remain unknown. We report the case of a 12-year-old boy who presented with fever for 4 days, swollen cervical lymph nodes, conjunctival congestion, red lips, strawberry tongue, irregular erythematous lesions, abdominal pain, and diarrhoea; he had coronavirus disease 2019 (COVID-19) 1 month prior. The patient was diagnosed MIS-C/MAS based on the criteria for sJIA/MAS, treated with intravenous immunoglobulin, glucocorticoids, and cyclosporine without any coronary artery sequelae. Evaluation of the patient's serum cytokine profile revealed that CXCL9 level (14259 pg/mL, reference range <31-83 pg/mL) was significantly elevated, as in sJIA/MAS. Based on cytokine profiles, the diagnostic criteria for MIC-S/MAS may be consistent with those for sJIA/MAS. No previous studies have reported on the cytokine profiles of MIS-C/MAS. Pediatricians should consider that MIS-C may complicate MAS, such as sJIA. Glucocorticoids and cyclosporine may be considered in cases of MIS-C complicated by MAS.
