RT Journal Article
SR Electronic
T1 Clinical Features of Hemophagocytic Syndrome in Patients with Dermatomyositis
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP 1838
OP 1841
VO 35
IS 9
A1 NOBUYUKI YAJIMA
A1 KUNINOBU WAKABAYASHI
A1 TSUYOSHI ODAI
A1 TAKEO ISOZAKI
A1 MIZUHO MATSUNAWA
A1 YUSUKE MIWA
A1 MASAO NEGISHI
A1 HIROTSUGU IDE
A1 TSUYOSHI KASAMA
YR 2008
UL http://www.jrheum.org/content/35/9/1838.abstract
AB Objective To investigate the clinical features of patients with dermatomyositis (DM) complicated by hemophagocytic syndrome (HPS). Methods Twenty-four patients diagnosed with DM and treated at our hospital between January 2002 and April 2007 were enrolled for study. Serum levels of various parameters including cytokines were determined during the active disease states. Results Levels of serum ferritin, creatine kinase, and immune complexes were all significantly higher in all patients with HPS than in those without HPS. Levels of soluble interleukin-2 receptor, macrophage colony stimulating factor, and the chemokine CX3CL1 were significantly elevated in DM patients with HPS. Conclusion Our findings suggest that mechanisms related to both circulating immune complexes and circulating cytokines are involved in the pathogenesis of HPS complicating DM.