title: Clinical significance of von Willebrand factor in patients with adult dermatomyositis.
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.
authors.affiliation: The Department of Medicine, The Tazuke Kofukai, Medical Research Institute, Kitano Hospital, Ohgimachi, Kita-ku, Osaka, Japan. t-komiya@kitano-hp.or.jp
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Komiya
authors.forename: Nobuo
authors.initials: N
authors.lastname: Negoro
authors.forename: Kyoko
authors.initials: K
authors.lastname: Kondo
authors.forename: Katsuyuki
authors.initials: K
authors.lastname: Miura
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Hirota
authors.forename: Junichi
authors.initials: J
authors.lastname: Yoshikawa
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D000369:Aged, 80 and over; D015415:Biomarkers / Q000097:blood; D016022:Case-Control Studies; D016001:Confidence Intervals; D003882:Dermatomyositis / Q000097:blood* / Q000175:diagnosis* / Q000453:epidemiology; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D011336:Probability; D012016:Reference Values; D018570:Risk Assessment; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index; D017678:Sex Distribution; D014841:von Willebrand Factor / Q000378:metabolism*
chemical_list: D015415:Biomarkers; D014841:von Willebrand Factor