抗合成酶综合征与临床免疫学家的定位

В. В. Чоп’як, Христина Ліщук-Якимович, Роман Пукаляк, Омелян Синенький
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摘要

抗合成酶综合征是一种临床和实验室综合征,发生于特发性炎性肌病患者,其特征是发展为间质性肺疾病,即纤维化肺泡炎综合征,对传统皮质激素治疗的抵抗以及存在肌炎特异性抗体。我们提出一个临床病例的抗合成酶综合征在中年患者谁已经提出了严重的肌痛综合征,光性皮肤病,雷诺现象。该病首发表现为皮肤(向日性红斑,上半身皮肤红斑疹)和肌痛症状,发热伴关节综合征,以及肺损伤(肺部炎和浸润)。免疫检测显示抗jo -1、抗pl -12、抗pl -7抗体(«Polycheck»,BIOCHECK,德国)。多年来,患者一直被诊断为抗合成酶综合征。使用环磷酰胺和甲基强的松龙联合脉冲治疗,以及添加高剂量维生素D3,有助于恢复损伤和减少皮肌炎活动。
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ANTISYNTHETASE SYNDROME AND THE POSITION OF CLINICAL IMMUNOLOGIST
Antisynthetase syndrome is a clinical and laboratory syndrome that develops in patients with idiopathic inflammatory myopathy and is characterized by the development of interstitial lung disease, namely fibrosing alveolitis syndrome, resistance to traditional corticoid therapy and the presence of myositis-specific antibodies.We present a clinical case of an antisynthetase syndrome in a middle-aged patient who has presented severe myalgic syndrome, photodermatosis, Raynaud’s phenomenon. The disease debuted with cutaneous (heliotropic erythema, erythematous rash on the skin of the upper torso) and myalgic symptoms, fever with next adding of the joint syndrome, as well as lung damage (pulmonitis and infiltrates). Immunological testing revealed anti-Jo-1, anti-PL-12-, anti-PL-7 antibodies («Polycheck», BIOCHECK, Germany). Since years, the patient has got the diagnosis of antisynthetase syndrome. The use of combined pulse therapy with cyclophosphamide and methylprednisolone, as well as the addition of high doses of vitamin D3 has contributed to the regression oflung damage and reduction of dermatomyositis activity.
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