title: Clinical features of hemophagocytic syndrome in patients with dermatomyositis.
abstract: 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.
  
  
  CONCLUSIONS
  Our findings suggest that mechanisms related to both circulating immune complexes and circulating cytokines are involved in the pathogenesis of HPS complicating DM.
authors.affiliation: Department of Internal Medicine, Showa University School of Medicine, Tokyo, Japan.
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Yajima
authors.forename: Kuninobu
authors.initials: K
authors.lastname: Wakabayashi
authors.forename: Tsuyoshi
authors.initials: T
authors.lastname: Odai
authors.forename: Takeo
authors.initials: T
authors.lastname: Isozaki
authors.forename: Mizuho
authors.initials: M
authors.lastname: Matsunawa
authors.forename: Yusuke
authors.initials: Y
authors.lastname: Miwa
authors.forename: Masao
authors.initials: M
authors.lastname: Negishi
authors.forename: Hirotsugu
authors.initials: H
authors.lastname: Ide
authors.forename: Tsuyoshi
authors.initials: T
authors.lastname: Kasama
mesh_terms: D000293:Adolescent; D000328:Adult; D000369:Aged, 80 and over; D000936:Antigen-Antibody Complex / Q000097:blood; D054428:Chemokine CX3CL1 / Q000097:blood; D003402:Creatine Kinase / Q000097:blood; D003882:Dermatomyositis / Q000097:blood / Q000175:diagnosis* / Q000276:immunology; D005293:Ferritins / Q000097:blood; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000097:blood / Q000175:diagnosis* / Q000276:immunology; D016173:Macrophage Colony-Stimulating Factor / Q000097:blood; D008297:Male; D008875:Middle Aged; D015375:Receptors, Interleukin-2 / Q000097:blood
chemical_list: D000936:Antigen-Antibody Complex; C515538:CX3CL1 protein, human; D054428:Chemokine CX3CL1; D015375:Receptors, Interleukin-2; D016173:Macrophage Colony-Stimulating Factor; D005293:Ferritins; D003402:Creatine Kinase