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Evaluation of Macrophage Activation Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis: A Single Center
Pia Elkjær Høeg1,2, Mia Glerup1, Birgitte Mahler1
1Pediatric Rheumatology Clinic, Department of Pediatrics and Adolescent Medicine, Aarhus University Hospital, Denmark.
Objectives:
Macrophage activation syndrome (MAS) is a severe complication of systemic juvenile arthritis (sJIA), and early diagnosis is critical for survival. The objective of this study was to evaluate the 2016 MAS classification criteria in a Danish sJIA cohort and to compare different sets of criteria for the early identification of MAS including the HLH-2004 diagnostic guidelines, MS score, and the ferritin/ESR ratio.
Methods:
Data was extracted from medical charts of 32 patients with sJIA from a single Danish paediatric rheumatology center diagnosed between January 2014 and June 2021. Patients who met the 2016 MAS classification criteria were classified as having MAS. From a receiver operating characteristic (ROC) plot, the area under the curve (AUC) was calculated for the prediction of patients with MAS according to the 2016 MAS classification criteria using either MS score or the ferritin/ESR ratio.
Results:
Of the cohort, eight (25%) patients were classified as having MAS according to the 2016 MAS classification criteria compared to only three (9.4%) patients fulfilling the HLH-2004 diagnostic guidelines, all of which had recurrent MAS. The ferritin/ESR ratio showed the highest sensitivity (100%) but the lowest specificity (72.2%). In comparison, the MS score had a higher specificity (90.9%) for the identification of MAS according to the 2016 classification criteria. In our cohort, the most optimal cut-off point for the ferritin/ESR ratio was ≥19.4 (sensitivity: 100%, specificity: 72.2%) and ≥ -1.5 for the MS score (sensitivity: 71.4%, specificity: 91.7%), respectively.
Conclusion:
The 2016 MAS classification criteria were a valuable tool in the discrimination of sJIA with and without MAS. The HLH-2004 diagnostic guidelines showed the lowest sensitivity, ferritin/ESR ratio, and the lowest specificity compared to the MS score where an acceptable high sensitivity and specificity was found.
Insights
The 2016 Macrophage Activation Syndrome (MAS) criteria effectively identified MAS in systemic juvenile idiopathic arthritis (sJIA) patients. The MS score demonstrated superior specificity compared to the ferritin/ESR ratio for early MAS detection.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Clinical Diagnostics
Background:
- Macrophage Activation Syndrome (MAS) is a life-threatening complication of systemic juvenile idiopathic arthritis (sJIA).
- Early and accurate diagnosis of MAS is crucial for patient survival.
- Evaluating diagnostic criteria is essential for improving clinical management.
Purpose of the Study:
- To assess the performance of the 2016 MAS classification criteria in a Danish sJIA cohort.
- To compare the efficacy of different criteria (HLH-2004, MS score, ferritin/ESR ratio) for early MAS identification.
Main Methods:
- Retrospective analysis of medical records from 32 sJIA patients diagnosed between 2014 and 2021.
- Classification of patients based on the 2016 MAS criteria.
- Receiver Operating Characteristic (ROC) curve analysis to evaluate the predictive accuracy of MS score and ferritin/ESR ratio.
Main Results:
- Eight patients (25%) met the 2016 MAS criteria; only three (9.4%) met HLH-2004 guidelines.
- The ferritin/ESR ratio had 100% sensitivity but 72.2% specificity (optimal cut-off ≥19.4).
- The MS score showed higher specificity (90.9% for 2016 criteria; optimal cut-off ≥-1.5) with 71.4% sensitivity.
Conclusions:
- The 2016 MAS classification criteria are valuable for distinguishing sJIA with and without MAS.
- The MS score offers a balance of acceptable sensitivity and high specificity for early MAS detection in sJIA.
- HLH-2004 guidelines demonstrated the lowest sensitivity in this cohort.
