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利用肺部超声 B 线和 KL-6 监测抗 MDA-5 抗体阳性的临床无肌病性皮肌炎相关间质性肺病:病例报告及文献复习。

Utilize lung ultrasound B-lines and KL-6 to monitor anti-MDA-5 antibody-positive clinically amyopathic dermatomyositis-associated interstitial lung disease: a case report and literature review.

机构信息

Department of Rheumatology and Immunology, Shantou Central Hospital, Shantou, Guangdong, China.

Department of Geriatric Medicine, Division of Rheumatology AOUC, University of Florence, Florence, Italy.

出版信息

Clin Rheumatol. 2019 May;38(5):1433-1436. doi: 10.1007/s10067-019-04462-z. Epub 2019 Feb 11.

Abstract

In the past decade, lung ultrasound (LUS) B-lines and serum Krebs von den Lungen-6 (KL-6) antigen have been recognized as biomarkers of the connective tissue disease-associated interstitial lung diseases (CTD-ILDs). Robust data have demonstrated that B-lines total numbers and KL-6 levels are correlated with high-resolution computed tomography findings, pulmonary function test, and some clinical parameters in CTD-ILDs. However, limited data are available regarding the use of these two biomarkers to follow CTD-ILDs. Herein, we report a case with anti-melanoma differentiation-associated gene 5 antibody-positive clinically amyopathic dermatomyositis-associated ILD, successfully treated with high-dose methylprednisolone, cyclophosphamide, intravenous immunoglobulin, pirfenidone, and followed using lung ultrasound and KL-6.

摘要

在过去十年中,肺部超声(LUS)B 线和血清 Krebs von den Lungen-6(KL-6)抗原已被认为是结缔组织病相关间质性肺疾病(CTD-ILD)的生物标志物。大量数据表明,B 线总数和 KL-6 水平与高分辨率计算机断层扫描结果、肺功能测试以及 CTD-ILD 的一些临床参数相关。然而,关于这两种生物标志物用于监测 CTD-ILD 的数据有限。在此,我们报告了一例抗黑色素瘤分化相关基因 5 抗体阳性的临床无肌病性皮肌炎相关间质性肺病,经大剂量甲基强的松龙、环磷酰胺、静脉注射免疫球蛋白、吡非尼酮治疗后成功,并用肺部超声和 KL-6 进行随访。

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