Tseng Chih-Wei, Wang Kao-Lun, Fu Pin-Kuei, Huang Cheng-Yi, Hsieh Tsu-Yi, Hsieh Chia-Wei, Lai Kuo-Lung, Hung Wei-Ting, Lin Ching-Tsai, Tang Kuo-Tung, Chen Yi-Ming, Huang Wen-Nan, Chen Yi-Hsing
Division of Allergy, Immunology and Rheumatology, Department of Internal Medicine, Taichung Veterans General Hospital, Taichung 40705, Taiwan.
Department of Radiology, Taichung Veterans General Hospital, Taichung 40705, Taiwan.
J Clin Med. 2021 Nov 11;10(22):5241. doi: 10.3390/jcm10225241.
Anti-melanoma differentiation-associated gene 5 (MDA-5) antibody is associated with respiratory failure and death in patients with idiopathic inflammatory myositis (IIM) and interstitial lung disease (ILD). This study aimed to investigate clinical parameters associated with mortality in anti-MDA-5 antibody-positive patients.
We retrospectively reviewed the clinical and laboratory data, and pulmonary function test results in 55 anti-MDA-5 antibody-positive patients. A comparison was made between the survivors and non-survivors at the 12-month follow-up.
A total of 13 patients (23.6%) died within 12 months. Non-survivors had higher GAP scores (gender, age, and physiology score for idiopathic pulmonary fibrosis) (1 vs. 6, < 0.01) and CA-153 (16.4 vs. 72.9, < 0.01). In addition, rapid progressive ILD, fever, peak ferritin, leukocyte count, lactate dehydrogenase, CT score, intravenous immunoglobulin, mycophenolic acid, CMV infections, pneumocystis pneumonia, and pneumothorax were significantly associated with increased risks of 1-year mortality, while forced vital capacity, forced expiratory volume in one second, and diffusion capacity for carbon monoxide were correlated with decreased risk of 1-year mortality.
Our study results suggest that GAP scores and CA-153 could be prognostic factors for 1-year mortality in anti-MDA-5 antibody-positive patients. A prompt pulmonary function test and CA-153 are essential for these patients to guide further management.
抗黑色素瘤分化相关基因5（MDA-5）抗体与特发性炎性肌病（IIM）和间质性肺病（ILD）患者的呼吸衰竭及死亡相关。本研究旨在调查抗MDA-5抗体阳性患者中与死亡率相关的临床参数。
我们回顾性分析了55例抗MDA-5抗体阳性患者的临床、实验室数据及肺功能测试结果。对12个月随访期内的存活者和非存活者进行了比较。
共有13例患者（23.6%）在12个月内死亡。非存活者的GAP评分（特发性肺纤维化的性别、年龄和生理学评分）更高（1对6，<0.01），CA-153水平也更高（16.4对72.9，<0.01）。此外，快速进展性ILD、发热、铁蛋白峰值、白细胞计数、乳酸脱氢酶、CT评分、静脉注射免疫球蛋白、霉酚酸、巨细胞病毒感染、肺孢子菌肺炎和气胸与1年死亡率风险增加显著相关，而用力肺活量、一秒用力呼气容积和一氧化碳弥散量与1年死亡率风险降低相关。
我们的研究结果表明，GAP评分和CA-153可能是抗MDA-5抗体阳性患者1年死亡率的预后因素。对这些患者进行及时的肺功能测试和CA-153检测对于指导进一步治疗至关重要。
