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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 in MIS-C
Luisa Berenise Gámez-González1, Chiharu Murata2, Jimena García-Silva3
1Department of Immunology, Hospital Infantil Especialidades de Chihuahua, Faculty of Medicine and Biological Sciences of the Autonomous University of Chihuahua, Chihuahua, México.
Background:
Multisystem inflammatory syndrome (MIS-C) represents a diagnostic challenge because of its overlap with Kawasaki disease, Kawasaki disease shock syndrome, and toxic shock syndrome. Macrophage activation syndrome (MAS) is a frequently fatal complication of various pediatric inflammatory disorders and has been reported in MIS-C. Early diagnosis and prompt initiation by immune modulating therapies are essential for effectively managing MAS.
Methods:
We conducted a retrospective study to determine the frequency, natural history, diagnostic metrics, treatment, and outcome of MAS in MIS-C within a large cohort of patients across 84 Latin American centers in 16 countries. We compared the clinical and laboratory characteristics between patients with and without MAS.
Results:
Among 1238 patients with MIS-C, 212 (17.1%) fulfilled MAS criteria. Gastrointestinal and neurologic manifestations were more frequent in cases where MIS-C was complicated by MAS. Patients presenting with MIS-C complicated by MAS had a mortality rate of 12%, which was higher than those without it. Mortality was associated with MAS, seizures, arthritis, and shock. A ferritin or erythrocyte sedimentation rate ratio of >18.7 exhibited a sensitivity of 88.2% and a specificity of 75% in diagnosing MAS in MIS-C.
Conclusions:
MAS in MIS-C patients is associated with increased morbidity and mortality rates in the largest MIS-C Latin American cohort. Early recognition and appropriate management are crucial in improving patient outcomes and reducing mortality rates.
Insights
Macrophage activation syndrome (MAS) complicates Multisystem Inflammatory Syndrome in children (MIS-C), increasing mortality. Early recognition and treatment are vital for better outcomes in MIS-C patients.
Area of Science:
- Pediatric rheumatology
- Infectious diseases
- Critical care medicine
Background:
- Multisystem Inflammatory Syndrome in children (MIS-C) presents diagnostic challenges due to overlapping symptoms with other severe pediatric conditions.
- Macrophage Activation Syndrome (MAS) is a severe, often fatal, complication of pediatric inflammatory disorders, increasingly reported in MIS-C.
- Timely diagnosis and immunomodulatory therapy are critical for managing MAS effectively.
Purpose of the Study:
- To investigate the frequency, clinical course, diagnostic markers, treatment, and outcomes of MAS in MIS-C patients.
- To compare clinical and laboratory features between MIS-C patients with and without MAS.
- To identify predictors of mortality in MIS-C patients with MAS.
Main Methods:
- Retrospective study across 84 Latin American centers involving 1238 MIS-C patients.
- MAS diagnosis was based on established criteria.
- Comparative analysis of clinical and laboratory data between MIS-C patients with and without MAS.
Main Results:
- 17.1% of MIS-C patients met MAS criteria.
- Gastrointestinal and neurological symptoms were more common in MIS-C with MAS.
- MIS-C with MAS had a 12% mortality rate, associated with seizures, arthritis, and shock.
- A ferritin or ESR ratio >18.7 showed 88.2% sensitivity and 75% specificity for MAS diagnosis.
Conclusions:
- MAS significantly increases morbidity and mortality in MIS-C patients.
- This study represents the largest Latin American cohort analyzing MAS in MIS-C.
- Prompt recognition and management are essential to improve survival rates in MIS-C patients with MAS.
