Systemic　lupus　erythematosus　associated　macrophage　activation　syndrome；a　case　report．

Abstract

　　　Macrophage　activation　syndrome（MAS）has　been　observed　in　patients　with　systemic　lupus　erythematosus（ SLE）．Recognition　of　MAS　in　patients　with　SLE　may　be　particularly　challenging　because　it　may　mimic the　clinical　features　of　the　underlying　disease　or　be　confused　with　an　infectious　complication．　We　present　a　case of　SLE　associated　MAS（SLE-MAS）．　The　patient　showed　the　distinct　cytokine　profile　of　SLE-MAS　compared to　systemic　juvenile　idiopathic　arthritis　associated　MAS．　The　observed　TNF-α dominant　increase　appears to　be　characteristic　of　SLE-MAS．IgM　type　antilymphocyte　antibody（ALAB）was　detected　on　the　surface of　lymphocytes　during　the　acute　phase　and　disappeared　when　the　patient　was　in　remission．　The　patient　had　a heterozygous　P369S-R408Q　mutation　in　the　MEFV　gene．　Our　results　suggest　that　ALAB　and　a　MEFV　mutation might　play　important　roles　in　the　pathogenesis　of　SLE-MAS．　Furthermore，the　cytokine　profile　of　SLE-MAS differs　from　that　of　S-JIA-MAS：the　TNF-α dominant　increase　appears　to　be　characteristic．