Dysregulation of Angiopoietin in systemic juvenile idiopathic arthritis associated macrophage activation syndrome.

Abstract

Angiopoictins（Ang）l　and　2　are　important　regulators　of　endothelial　cell　function．　To　assess　the　role　of Ang-l　and　2　dysregulation　in　the　pathogenesis　of　systemic　juvenile　idiopathic　arthritis（s-JIA）associated macrophage　activation　syndrome（MAS），　and　to　investigate　clinical　significance　of serum　Ang　2/l　ratio　in　s-JIA/ MAS，　we　measured　serum　Ang　l/2 1evels　by　enzyme-linked　immunosorbent　assay．　Twenty five　patients　with s-JIA　including　10　patients　with　MAS，　11　patients　with　polyarticular　JIA　and　15　healthy　children　were　analyzed． Results　were　compared　with　clinical　features　of　s-JIA．　Serum　Ang－l　levels　during　MAS　phase　were　significantly decreased　compared　to　those　during　acute　phase　in　s-JIA　patients．　Serum　Ang　2/1　ratio　during　MAS　phase　were significantly　elevated　compared　to　those　during　acute　phase　in　s-JIA　patients．　The　Ang　2／l　ratio　was　rapidly　and extremely　elevated　during　MAS　phase，　Ang　2/l　ratio　was　also　rapidly　decreased　after　treatment．　The　Ang　2/l ratio　correlated　positively　with　LDH　and　FDP-D　dimer．　Serum　Ang　2/l　ratio　levels　to　reflect　endothelial　damage might　be　a　promising　indicator　of disease　activity　for　s-JIA/MAS．