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Author Spotlight: Isolation and Culture of Primary Synovial Macrophages and Fibroblasts from Murine Arthritis Tissue
Published on: February 24, 2023
Single center clinical analysis of macrophage activation syndrome complicating juvenile rheumatic diseases
Shuoyin Huang1, Yingying Liu1, Wu Yan2
1Department of Rheumatology and Immunology, Children's Hospital of Nanjing Medical University, Nanjing, 210008, China.
Background:
Macrophage activation syndrome (MAS), an example of secondary hemophagocytic lymphohistiocytosis, is a potentially fatal complication of rheumatic diseases. We aimed to study the clinical and laboratory characteristics, treatment schemes, and outcomes of different rheumatic disorders associated with MAS in children. Early warning indicators of MAS have also been investigated to enable clinicians to make a prompt and accurate diagnosis.
Methods:
Fifty-five patients with rheumatic diseases complicated by MAS were enrolled between January 2017 and December 2022. Clinical and laboratory data were collected before disease onset, at diagnosis, and after treatment with MAS, and data were compared between patients with systemic juvenile idiopathic arthritis (sJIA), Kawasaki disease (KD), and systemic lupus erythematosus (SLE). A random forest model was established to show the importance score of each variable with a significant difference.
Results:
Most (81.8%) instances of MAS occurred during the initial diagnosis of the underlying disease. Compared to the active stage of sJIA, the platelet count, erythrocyte sedimentation rate, and fibrinogen level in sJIA-MAS were significantly decreased, whereas ferritin, ferritin/erythrocyte sedimentation rate, aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase, and D-dimer levels were significantly increased. Ferritin level, ferritin/erythrocyte sedimentation rate, and platelet count had the greatest predictive value for sJIA-MAS. The level of IL-18 in the sJIA-MAS group was significantly higher than in the active sJIA group, whereas IL-6 levels were significantly lower. Most patients with MAS were treated with methylprednisolone pulse combined with cyclosporine, and no deaths occurred.
Conclusions:
Thrombocytopenia, ferritin levels, the ferritin/erythrocyte sedimentation rate, and elevated aspartate aminotransferase levels can predict the occurrence of MAS in patients with sJIA. Additionally, our analysis indicates that IL-18 plays an important role in the pathogenesis of MAS in sJIA-MAS.
Insights
Macrophage activation syndrome (MAS) in children with rheumatic diseases is often diagnosed early. Specific markers like ferritin and IL-18 can predict MAS in systemic juvenile idiopathic arthritis (sJIA).
Area of Science:
- Pediatric Rheumatology
- Immunology
- Hematology
Background:
- Macrophage activation syndrome (MAS), a severe complication of rheumatic diseases, requires early diagnosis.
- Investigating MAS in children with systemic juvenile idiopathic arthritis (sJIA), Kawasaki disease (KD), and systemic lupus erythematosus (SLE) is crucial.
- Identifying early warning indicators for MAS can improve prompt diagnosis and management.
Purpose of the Study:
- To analyze clinical and laboratory features of MAS in children with rheumatic disorders.
- To compare MAS characteristics across different rheumatic diseases, specifically sJIA, KD, and SLE.
- To identify predictive indicators for MAS development in pediatric rheumatic diseases.
Main Methods:
- A retrospective study of 55 pediatric patients with rheumatic diseases complicated by MAS (Jan 2017-Dec 2022).
- Collection and comparison of clinical and laboratory data before MAS onset, at diagnosis, and post-treatment.
- Utilized a random forest model to identify significant predictive variables for MAS.
Main Results:
- MAS frequently occurred at the initial diagnosis of the underlying rheumatic disease (81.8%).
- In sJIA-MAS, decreased platelets, ESR, and fibrinogen, with increased ferritin, ferritin/ESR, AST, ALT, LDH, and D-dimer were observed.
- Ferritin, ferritin/ESR, and platelet count showed high predictive value for sJIA-MAS; IL-18 was elevated, and IL-6 was lower in sJIA-MAS.
Conclusions:
- Thrombocytopenia, elevated ferritin, ferritin/ESR ratio, and AST predict MAS in sJIA patients.
- Interleukin-18 (IL-18) is implicated in the pathogenesis of MAS in sJIA.
- Treatment with methylprednisolone pulse and cyclosporine resulted in no deaths among MAS patients.
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