Komiya Toshiyuki, Negoro Nobuo, Kondo Kyoko, Miura Katsuyuki, Hirota Yoshio, Yoshikawa Junichi
The Department of Medicine, The Tazuke Kofukai, Medical Research Institute, Kitano Hospital, Ohgimachi, Kita-ku, Osaka, Japan.
Clin Rheumatol. 2005 Aug;24(4):352-7. doi: 10.1007/s10067-004-1027-z. Epub 2004 Nov 23.
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.
由于血管性血友病因子（vWF）作为内皮损伤的标志物，其在成年皮肌炎（DM）患者中的临床意义尚未得到充分研究，因此我们评估了血浆vWF水平是否可作为这些患者疾病活动度的指标。我们采用酶联免疫吸附测定法测量了11例成年活动性DM患者、13例非活动性DM患者和18名健康受试者的血浆vWF抗原水平。使用协方差分析和逻辑回归分析研究了DM患者中vWF水平与临床状况及肌肉源性酶泄漏之间的关联。此外，我们研究了治疗对vWF抗原水平的影响。活动性DM患者的平均vWF抗原水平显著高于非活动性DM患者和健康受试者。较高的vWF水平与全身乏力、发热和肌肉无力等临床症状相关。它们还与天冬氨酸转氨酶、丙氨酸转氨酶和醛缩酶水平相关，但与乳酸脱氢酶和肌酸激酶（CK）水平无关。vWF抗原与除CK外的肌肉酶相关。6例活动性DM患者在成功接受皮质类固醇治疗后血浆vWF水平显著下降。血浆vWF水平可被视为成年DM患者疾病活动度的有用标志物。
