PT - JOURNAL ARTICLE AU - NOBUYUKI YAJIMA AU - KUNINOBU WAKABAYASHI AU - TSUYOSHI ODAI AU - TAKEO ISOZAKI AU - MIZUHO MATSUNAWA AU - YUSUKE MIWA AU - MASAO NEGISHI AU - HIROTSUGU IDE AU - TSUYOSHI KASAMA TI - Clinical Features of Hemophagocytic Syndrome in Patients with Dermatomyositis DP - 2008 Sep 01 TA - The Journal of Rheumatology PG - 1838--1841 VI - 35 IP - 9 4099 - http://www.jrheum.org/content/35/9/1838.short 4100 - http://www.jrheum.org/content/35/9/1838.full SO - J Rheumatol2008 Sep 01; 35 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.