Clinical features of hemophagocytic syndrome in patients with dermatomyositis.
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.
Yajima
Nobuyuki
N
Department of Internal Medicine, Showa University School of Medicine, Tokyo, Japan.
Wakabayashi
Kuninobu
K
Odai
Tsuyoshi
T
Isozaki
Takeo
T
Matsunawa
Mizuho
M
Miwa
Yusuke
Y
Negishi
Masao
M
Ide
Hirotsugu
H
Kasama
Tsuyoshi
T
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
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