Clinical Features of Hemophagocytic Syndrome in Patients with Dermatomyositis

NOBUYUKI YAJIMA, KUNINOBU WAKABAYASHI, TSUYOSHI ODAI, TAKEO ISOZAKI, MIZUHO MATSUNAWA, YUSUKE MIWA, MASAO NEGISHI, HIROTSUGU IDE, and TSUYOSHI KASAMA

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.

Conclusion. Our findings suggest that mechanisms related to both circulating immune complexes and circulating cytokines are involved in the pathogenesis of HPS complicating DM. (First Release July 15 2008; J Rheumatol 2008;35:1838–41)

Key Indexing Terms:

HEMOPHAGOCYTIC SYNDROME
DERMATOMYOSITIS
CYTOKINE
CX3CL1

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From the Division of Rheumatology, Department of Internal Medicine, Showa University School of Medicine, Tokyo, Japan.

N. Yajima, MD; K. Wakabayashi, MD; T. Odai, MD; T. Isozaki, MD; M. Matsunawa, MD; Y. Miwa, MD; M. Negishi, MD; H. Ide, MD; T. Kasama, MD, Division of Rheumatology, Department of Internal Medicine, Showa University School of Medicine.

Address reprint requests to Dr. T. Kasama, Division of Rheumatology, Department of Internal Medicine, Showa University School of Medicine, Tokyo, 142-8666, Japan. E-mail: tkasama@med.showa-u.ac.jp

Accepted for publication April 2, 2008.

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