Characteristic elevation of soluble TNF receptor II : I ratio in macrophage activation syndrome with systemic...

Area of Science:

• Immunology
• Pediatric Rheumatology
• Hematology

Background:

• Macrophage activation syndrome (MAS) is a life-threatening complication of systemic juvenile idiopathic arthritis (s-JIA).
• Accurate diagnosis and differentiation from other inflammatory conditions like Epstein-Barr virus-induced haemophagocytic lymphohistiocytosis (EBV-HLH) are crucial.
• Current diagnostic markers for MAS require further validation, especially in pediatric populations.

Purpose of the Study:

• To evaluate the clinical significance of the soluble tumor necrosis factor receptor (sTNF-R) II/I ratio for diagnosing MAS in s-JIA.
• To assess the correlation of sTNF-RII/I ratio with disease activity and severity in s-JIA patients.
• To compare serum interleukin (IL)-18 levels between MAS and EBV-HLH patients.

Main Methods:

• Serum sTNF-RI and sTNF-RII levels were measured in 117 s-JIA patients (29 with MAS, 15 with EBV-HLH, 15 with Kawasaki disease, 28 healthy controls).
• Disease activity and severity measurements were correlated with sTNF-RII/I ratios.
• Serum IL-18 levels were measured in MAS and EBV-HLH patients.

Main Results:

• The sTNF-RII/I ratio was significantly elevated in MAS and EBV-HLH patients compared to acute s-JIA and Kawasaki disease patients.
• No significant difference in sTNF-RII/I ratio was observed between healthy controls and acute s-JIA patients.
• The sTNF-RII/I ratio increased profoundly with MAS development and positively correlated with disease activity.
• Serum IL-18 levels were significantly higher in MAS patients than in EBV-HLH patients.

Conclusions:

• The sTNF-RII/I ratio is a valuable indicator for diagnosing MAS in s-JIA.
• Monitoring serum IL-18 and sTNF-RII/I ratios may aid in differentiating MAS from EBV-HLH.
• These biomarkers offer potential for improved diagnostic accuracy and patient management in complex pediatric inflammatory conditions.