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Clinical significance of von Willebrand factor in patients with adult dermatomyositis

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Abstract

Because the clinical significance of von Willebrand factor (vWF), a marker of endothelial injury, has not been well studied in adult patients with dermatomyositis (DM), we evaluated whether plasma vWF levels are useful as an index of disease activity in these patients. We measured plasma vWF antigen levels in 11 patients with active adult DM, 13 patients with inactive DM, and 18 healthy subjects using an enzyme-linked immunosorbent assay. The association of vWF level with clinical condition and muscle-derived enzyme leakage among DM patients was examined using analysis of covariance and logistic regression analysis. Furthermore, we studied the effects of treatment on the vWF antigen level. The mean vWF antigen level was significantly higher in active DM patients than in inactive DM patients and healthy subjects. Higher vWF levels were associated with clinical symptoms, such as general fatigue, fever, and muscle weakness. They were also associated with the levels of aspartate aminotransferase, alanine aminotransferase, and aldolase, but not with those of lactate dehydrogenase and creatine kinase (CK). vWF antigen was correlated with muscle enzymes except for CK. The plasma vWF levels in six patients with active DM significantly decreased after successful corticosteroid treatment. Plasma vWF level may be considered a useful marker of disease activity in adult DM patients.

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Abbreviations

vWF:

von Willebrand factor

DM:

Dermatomyositis

PM:

Polymyositis

JDM:

Juvenile dermatomyositis

AST:

Aspartate aminotransferase

ALT:

Alanine aminotransferase

CK:

Creatine kinase

LDH:

Lactate dehydrogenase

ANCOVA:

Analysis of covariance

OR:

Odds ratio

CI:

Confidence interval

ELISA:

Enzyme-linked immunosorbent assay

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Acknowledgments

We are indebted to Dr. Mikio Okamura, Dr. Tomohiko Sumi, Dr. Jin Tsukamoto and Dr. Mizue Okazaki for their help in the collection of plasma samples, and to Mrs. Naomi Ishizawa for her technical assistance in measuring vWF antigen level.

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Authors and Affiliations

  1. The Department of Medicine, The Tazuke Kofukai, Medical Research Institute, Kitano Hospital, 2-4-20, Ohgimachi, Kita-ku, Osaka, Japan, 530-8480

    Toshiyuki Komiya

  2. The Department of Rheumatology and Nephrology, Osaka City University Medical School, Osaka, Japan

    Nobuo Negoro

  3. The Department of Public Health, Osaka City University Medical School, Osaka, Japan

    Kyoko Kondo & Yoshio Hirota

  4. The Department of Applied Pharmacology and Therapeutics, Osaka City University Medical School, Osaka, Japan

    Katsuyuki Miura

  5. The Department of Cardiology, Osaka City University Medical School, Osaka, Japan

    Junichi Yoshikawa

Authors
  1. Toshiyuki Komiya
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  2. Nobuo Negoro
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  3. Kyoko Kondo
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  4. Katsuyuki Miura
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  5. Yoshio Hirota
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  6. Junichi Yoshikawa
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Correspondence to Toshiyuki Komiya.

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Komiya, T., Negoro, N., Kondo, K. et al. Clinical significance of von Willebrand factor in patients with adult dermatomyositis. Clin Rheumatol 24, 352–357 (2005). https://doi.org/10.1007/s10067-004-1027-z

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  • DOI: https://doi.org/10.1007/s10067-004-1027-z

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