Göttfried I, Seeber A, Anegg B, Rieger A, Stingl G, Volc-Platzer B
Division of Immunology, Allergy and Infectious Diseases, Department of Dermatology, University of Vienna, Austria.
Eur J Dermatol. 2000 Jan-Feb;10(1):29-35.
An open study was conducted to identify and investigate dermatomyositis patients who benefit from IVIG treatment, based on dermatological criteria, myositis-related symptoms and immune/inflammatory parameters. 19 patients (16 females and three males, ages 31-84) suffered from dermatomyositis, and 4/19 patients had paraneoplastic dermatomyositis. We monitored the disease activity by documenting the clinical symptoms, recording muscle-related parameters (electromyography, serum creatine kinase, histopathology), and by determining circulating autoantibodies and serum levels of IL-6, sIL-2R, sTNF-a-R, sICAM-1, and sCD8. 7/19 patients responded to IVIG. They had severe skin but only moderate muscle involvement, no autoantibodies, and no malignancy. IVIG-nonresponders had severe skin and muscle disease, concomitant with autoantibodies and/or malignancy. sIL-2R levels were initially elevated in all patients but reverted to normal in IVIG-responders only. Creatine kinase-levels and other parameters did not correlate with disease activity and/or treatment response. IVIG is effective in selected dermatomyositis patients. sIL-2R serum levels appear to be useful predictors of IVIG-induced treatment response and disease activity.
开展了一项开放性研究，旨在基于皮肤病学标准、肌炎相关症状以及免疫/炎症参数，识别并研究从静脉注射免疫球蛋白（IVIG）治疗中获益的皮肌炎患者。19例患者（16例女性，3例男性，年龄31 - 84岁）患有皮肌炎，其中4/19例患者患有副肿瘤性皮肌炎。我们通过记录临床症状、记录肌肉相关参数（肌电图、血清肌酸激酶、组织病理学）以及测定循环自身抗体和血清白细胞介素-6（IL-6）、可溶性白细胞介素-2受体（sIL-2R）、可溶性肿瘤坏死因子-α受体（sTNF-a-R）、可溶性细胞间黏附分子-1（sICAM-1）和可溶性CD8水平来监测疾病活动。19例患者中有7例对IVIG有反应。他们皮肤病变严重但肌肉受累程度仅为中度，无自身抗体，也无恶性肿瘤。IVIG无反应者有严重的皮肤和肌肉疾病，伴有自身抗体和/或恶性肿瘤。所有患者的sIL-2R水平最初均升高，但仅在IVIG反应者中恢复正常。肌酸激酶水平和其他参数与疾病活动和/或治疗反应无关。IVIG对部分皮肌炎患者有效。sIL-2R血清水平似乎是IVIG诱导的治疗反应和疾病活动的有用预测指标。
