Increased circulating endothelial microparticles in children with FMF

Area of Science:

• Pediatric Rheumatology
• Vascular Biology
• Inflammatory Diseases

Background:

• Endothelial microparticles (EMPs) are recognized indicators of endothelial dysfunction.
• Familial Mediterranean fever (FMF) is a genetic autoinflammatory disorder.
• Endothelial dysfunction may be associated with inflammatory conditions.

Purpose of the Study:

• To investigate endothelial dysfunction in children diagnosed with FMF.
• To determine if endothelial dysfunction is more pronounced during active FMF attacks.
• To explore the relationship between EMP levels and disease activity in FMF.

Main Methods:

• A cross-sectional study involving 65 FMF patients (attack-free and attack periods) and 35 healthy controls.
• Measurement of circulating EMPs (CD144+, CD146+) via flow cytometry.
• Assessed serum amyloid A (SAA) and other inflammation markers, including C-reactive protein (CRP).

Main Results:

• Significantly elevated levels of CD144+ and CD146+ EMPs were observed in FMF patients during attack periods compared to controls.
• Inflammation markers were significantly higher in the FMF attack group versus control and attack-free groups.
• CD144+ and CD146+ EMP levels correlated significantly with CRP in FMF patients during attacks.

Conclusions:

• Endothelial damage is evident in children with FMF, especially during active disease phases.
• Endothelial dysfunction in FMF appears to be directly associated with the inflammatory state.
• EMPs serve as potential biomarkers for endothelial dysfunction in pediatric FMF.