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The Multifaceted Presentation of the Multisystem Inflammatory Syndrome in Children: Data from a Cluster Analysis
Hafize Emine Sönmez1, Şengül Çağlayan2, Gülçin Otar Yener3
1Department of Pediatric Rheumatology, Kocaeli University, Kocaeli 41001, Turkey.
Insights
Multisystem inflammatory syndrome in children (MIS-C) presents with diverse phenotypes, not a single disease. Identifying these distinct groups and their prognostic factors is crucial for individualized patient management and improved outcomes.
Area of Science:
- Pediatric Rheumatology
- Infectious Diseases
- Critical Care Medicine
Background:
- Multisystem inflammatory syndrome in children (MIS-C) is a serious condition requiring a deeper understanding of its varied presentations.
- Phenotypic variability in MIS-C suggests it may not be a monolithic disease, necessitating classification for effective treatment.
Purpose of the Study:
- To evaluate MIS-C patient outcomes based on distinct disease phenotypes.
- To identify prognostic factors associated with severe courses of MIS-C.
Main Methods:
- A cross-sectional study involving 293 MIS-C patients from seven pediatric rheumatology centers.
- Two-step cluster analysis was employed to categorize patients into distinct subgroups based on clinical features.
- Outcomes and laboratory findings were compared across identified patient clusters.
Main Results:
- Four MIS-C subgroups were identified: Kawasaki-like, MAS-like, LV dysfunction, and other presentations.
- The MAS-like and LV dysfunction groups experienced longer fever duration and hospitalization.
- Severe MIS-C cases showed elevated inflammatory markers, neutrophil-lymphocyte ratio, and cardiac biomarkers, with decreased lymphocyte and platelet counts.
Conclusions:
- MIS-C exhibits a spectrum of clinical features and outcomes, underscoring its heterogeneity.
- Recognizing these distinct phenotypes is essential for tailoring individualized management strategies in pediatric patients.
- Phenotypic classification aids in predicting disease severity and guiding therapeutic interventions.
Background:
The aim of this study was to evaluate the outcomes of patients with the multisystem inflammatory syndrome in children (MIS-C) according to phenotypes of disease and define the prognostic factors for the severe course.
Methods:
This cross-sectional study included 293 patients with MIS-C from seven pediatric rheumatology centers. A two-step cluster analysis was performed to define the spectrum of disease and their outcomes were compared between each group.
Results:
Four subgroups were identified as follows: cluster I, predominantly Kawasaki-like features (n = 100); cluster II, predominantly MAS-like features (n = 34); cluster III, predominantly LV dysfunction (n = 47); cluster IV, other presentations (n = 112). The duration of fever was longer in cluster II and the length of hospitalization was longer in both clusters II and III. Laboratory findings revealed lower lymphocyte and platelet counts and higher acute phase reactants (APRs) in cluster II, while patients in cluster IV showed less inflammation with lower APRs. The resolution of abnormal laboratory findings was longer in clusters II and III, while it was shortest in cluster IV. Seven patients died. Among them, four belonged to cluster II, while three were labeled as cluster III. Patients with severe course had higher levels of neutrophil-lymphocyte ratio, mean platelet volume, procalcitonin, ferritin, interleukin-6, fibrinogen, D-Dimer, BNP, and troponin-I, and lower levels of lymphocyte and platelet counts.
Conclusion:
As shown, MIS-C is not a single disease presenting with various clinical features and outcomes. Understanding the disease spectrum will provide individualized management.

