Clinical features of hemophagocytic syndrome in patients with dermatomyositis

J Rheumatol. 2008 Sep;35(9):1838-41. Epub 2008 Jul 15.


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.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Aged, 80 and over
  • Antigen-Antibody Complex / blood
  • Chemokine CX3CL1 / blood
  • Creatine Kinase / blood
  • Dermatomyositis / blood
  • Dermatomyositis / diagnosis*
  • Dermatomyositis / immunology
  • Ferritins / blood
  • Humans
  • Lymphohistiocytosis, Hemophagocytic / blood
  • Lymphohistiocytosis, Hemophagocytic / diagnosis*
  • Lymphohistiocytosis, Hemophagocytic / immunology
  • Macrophage Colony-Stimulating Factor / blood
  • Male
  • Middle Aged
  • Receptors, Interleukin-2 / blood


  • Antigen-Antibody Complex
  • CX3CL1 protein, human
  • Chemokine CX3CL1
  • Receptors, Interleukin-2
  • Macrophage Colony-Stimulating Factor
  • Ferritins
  • Creatine Kinase