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 parameter...

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Bibliographic Details
Published in:Journal of rheumatology 2008-09, Vol.35 (9), p.1838-1841
Main Authors: YAJIMA, Nobuyuki, WAKABAYASHI, Kuninobu, ODAI, Tsuyoshi, ISOZAKI, Takeo, MATSUNAWA, Mizuho, MIWA, Yusuke, NEGISHI, Masao, IDE, Hirotsugu, KASAMA, Tsuyoshi
Format: Article
Language:English
Subjects:
Adolescent
Adult
Aged, 80 and over
Antigen-Antibody Complex - blood
Biological and medical sciences
Chemokine CX3CL1 - blood
Creatine Kinase - blood
Dermatomyositis - blood
Dermatomyositis - diagnosis
Dermatomyositis - immunology
Diseases of the osteoarticular system
Ferritins - blood
Humans
Lymphohistiocytosis, Hemophagocytic - blood
Lymphohistiocytosis, Hemophagocytic - diagnosis
Lymphohistiocytosis, Hemophagocytic - immunology
Macrophage Colony-Stimulating Factor - blood
Male
Medical sciences
Middle Aged
Receptors, Interleukin-2 - blood
Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
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Summary: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. Key Indexing Terms: HEMOPHAGOCYTIC SYNDROME DERMATOMYOSITIS CYTOKINE CX3CL1
ISSN:0315-162X
1499-2752