Is it possible to predict a disease course prone to macrophage activation syndrome at systemic juvenile idiopathic
Ezgi Deniz Batu1, Seher Sener2, Zeynep Balık2
1Department of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, 06100, Ankara, Turkey. ezgidenizbatu@yahoo.com.
Objectives:
Macrophage activation syndrome (MAS) is a severe complication of systemic juvenile idiopathic arthritis (SJIA). We aimed to compare the characteristics of SJIA patients who developed MAS in the disease course to those who never experienced MAS.
Methods:
Patients with SJIA were included. The features of the patients at the time of SJIA diagnosis were compared. Multivariate logistic regression and ROC analyses were used while evaluating factors associated with MAS.
Results:
Overall, 126 SJIA patients (M/F:1.17) were included. Eighty-six (68.2%) never had MAS. At the time of SJIA diagnosis, age was younger; the duration of fever was longer; rash, hepatomegaly, and splenomegaly were more frequent and arthralgia/arthritis was less common among patients who had MAS in the follow-up than those who never had MAS. Also, white blood cell, neutrophil, and platelet counts and fibrinogen were lower, while transaminases, lactate dehydrogenase, triglyceride (TG), and ferritin levels were higher among patients with MAS than those without MAS. The multivariate regression analysis disclosed age at symptom onset, duration of fever, platelet count, TG and ferritin levels as independent MAS predictors. For ferritin level/platelet count (F/P) ratio at the time of SJIA diagnosis, a threshold of ≥1.1 performed best to predict a MAS-prone disease course with a sensitivity of 90% and a specificity of 82.6%.
Conclusion:
The F/P ratio at the time of SJIA diagnosis may be a promising biomarker to predict MAS-prone disease course in SJIA. Determining MAS-prone patients at the time of SJIA diagnosis could assist physicians while tailoring SJIA treatment individually. Key points • Systemic juvenile idiopathic arthritis (SJIA) patients with macrophage activation syndrome (MAS) differ from SJIA patients who never have MAS, at the time of SJIA diagnosis. • It could be possible to predict a MAS-prone disease course at the time of SJIA diagnosis. • The ferritin/platelet ratio is a promising biomarker for predicting MAS-prone SJIA disease course.
Insights
The ferritin/platelet ratio at diagnosis can predict macrophage activation syndrome (MAS) in systemic juvenile idiopathic arthritis (SJIA) patients. Early identification of MAS-prone SJIA aids personalized treatment strategies.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Hematology
Background:
- Macrophage activation syndrome (MAS) is a life-threatening complication of systemic juvenile idiopathic arthritis (SJIA).
- Identifying SJIA patients at risk for MAS is crucial for timely intervention and improved outcomes.
Purpose of the Study:
- To compare clinical and laboratory characteristics of SJIA patients who develop MAS versus those who do not.
- To identify predictors of MAS development in SJIA patients at the time of diagnosis.
Main Methods:
- Retrospective analysis of 126 SJIA patients.
- Comparison of patient features at SJIA diagnosis.
- Multivariate logistic regression and ROC analyses to identify MAS predictors.
Main Results:
- SJIA patients who developed MAS had distinct features at diagnosis, including younger age, longer fever duration, and specific laboratory findings.
- The ferritin/platelet (F/P) ratio emerged as a significant predictor of MAS.
- An F/P ratio threshold of ≥1.1 demonstrated high sensitivity (90%) and specificity (82.6%) for predicting a MAS-prone course.
Conclusions:
- The F/P ratio at SJIA diagnosis is a promising biomarker for predicting MAS development.
- Early identification of MAS-prone SJIA patients enables individualized treatment planning.
- This finding can help clinicians tailor therapies to mitigate MAS risk in SJIA.
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