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

• DOI: 10.1007/s10067-019-04462-z
• Publication Date: 2019-02-11T17:23:26Z
• Authors: Yukai Wang; Shaoqi Chen; Zhangzhang Lin; Jianqun Lin; Xuezhen Xie; Qisheng Lin; Guangzhou Du; Xiufeng Huang; Marco Matucci-Cer...; Daniel E Furst
• Keywords: Adult; Interferon-Induced Helicase, IFIH1; Mucin-1; Dermatomyositis; Respiratory Function Tests; 3. Good health; 03 medical and health sciences; 0302 clinical medicine; Antibodies, Antinuclear; Disease Progression; Humans; Female; Lung Diseases, Interstitial; Lung; Biomarkers; Ultrasonography

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.