Identification of the best cut-off points and clinical signs specific for early recognition of macrophage activation syndrome in active systemic juvenile idiopathic arthritis

Mikhail M Kostik,Olga Kalashnikova,Natalia Buchinskaya,Eugenia Isupova,Tatyana Kornishina,Vera Masalova,Vyacheslav Chasnyk,Ludmila Snegireva,Natalia Glebova,Eugenia Balbotkina,Tatyana Likhacheva,Ekaterina Kuchinskaya,Margarita Dubko,Irina Chikova

doi:10.1186/1546-0096-12-s1-p213

Abstract

Macrophage activation syndrome (MAS) – is a severe life-threatening hematological condition, mostly complicated systemic juvenile idiopathic arthritis (SJIA). Early detection of MAS can lead to appropriate therapeutic interventions and change the outcomes. There are no strict criteria for early MAS detection in SJIA. Currently applied HLH criteria can determinate only advanced stage of MAS, which lead to delay diagnosis, late start of specific treatment and associated with poor outcomes. There are several sets of preliminary criteria of MAS in SJIA.

Highlights

Macrophage activation syndrome (MAS) – is a severe lifethreatening hematological condition, mostly complicated systemic juvenile idiopathic arthritis (SJIA)
Our retrospective study was based on reviewing the medical charts of the children, admitted to the rheumatology department with active SJIA and definite MAS (n=18) and without MAS (n=40)
We evaluated the data related to SJIA and MAS at the moment of the patient’s admission

Summary

Introduction

Macrophage activation syndrome (MAS) – is a severe lifethreatening hematological condition, mostly complicated systemic juvenile idiopathic arthritis (SJIA). Detection of MAS can lead to appropriate therapeutic interventions and change the outcomes. There are no strict criteria for early MAS detection in SJIA. Applied HLH criteria can determinate only advanced stage of MAS, which lead to delay diagnosis, late start of specific treatment and associated with poor outcomes. There are several sets of preliminary criteria of MAS in SJIA

Objectives

Methods

Results