Serum KL-6 level is a prognostic marker in patients with anti-MDA5 antibody-positive dermatomyositis associated with interstitial lung disease
Serum KL-6 level is a prognostic marker in patients with anti-MDA5 antibody-positive dermatomyositis associated with interstitial lung disease
复制标题
血清 KL-6 水平是抗 MDA5 抗体阳性皮肌炎伴间质性肺病患者的预后标志物
DOI：
10.1002/jcla.22978
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发表时间：
2019-07-13
影响因子：
2.7
通讯作者：
Bao, Chunde
中科院分区：
文献类型：
--
作者：
Ye, Yan;Fu, Qiong;Bao, Chunde
目的探讨血清Krebs von den Lungen-6（KL-6）水平在抗-MDA 5抗体阳性皮肌炎（anti-MDA 5（+）DM）合并间质性肺病（ILD）患者，尤其是无肌病型DM患者中的临床意义。方法采用化学发光酶免疫分析法（CLEIA）检测抗MDA 5（+）DM患者（包括临床型无肌病性皮肌炎（CADM）-ILD和经典型DM-ILD）及健康体检者血清KL-6水平。在39例发生缓解或急性加重的CADM-ILD患者中测定基线和治疗后血清KL-6水平。分析了实验室检查结果、高分辨率计算机断层扫描（HRCT）评分、肺功能检查（PFT）和基线KL-6水平对死亡的预测值之间的关联。结果CADM-ILD患者血清KL-6水平（1339 ± 1329 U/mL）显著高于DM-ILD（642.3 ± 498.4 U/mL）和健康献血员（162.4 ± 54.01 U/mL）。KL-6水平与胸部HRCT评分、血清乳酸脱氢酶、血清铁蛋白水平和PFT呈正相关，但与红细胞沉降率无关。在随访期间，缓解/稳定组治疗后血清KL-6水平显著降低，而急性加重组治疗后血清KL-6水平升高。高水平的铁蛋白、KL-6和HRCT评分与不良预后独立相关。KL-6高表达组1年生存率明显低于KL-6低表达组。结论血清KL-6水平可作为抗-MDA 5（+）DM患者，尤其是无肌型DM患者ILD的预测和监测指标。
Objective This study aimed to investigate the clinical significance of Krebs von den Lungen-6 (KL-6) serum levels in patients with anti-MDA5 antibody-positive dermatomyositis (anti-MDA5(+) DM) having interstitial lung disease (ILD), especially in the amyopathic DM phenotype. Methods The serum KL-6 level was measured using a chemiluminescence enzyme immunoassay (CLEIA) in patients with anti-MDA5(+) DM, including clinically amyopathic dermatomyositis (CADM)-ILD and classic DM-ILD, and healthy donors. The baseline and post-treatment serum KL-6 levels were determined in 39 patients with CADM-ILD who experienced remission or acute exacerbation. The association between laboratory findings, high-resolution computed tomography (HRCT) scores, pulmonary function tests (PFTs), and the predictive value of baseline KL-6 level for death was analyzed. Results The serum KL-6 levels were significantly higher in patients with CADM-ILD (1339 +/- 1329 U/mL) compared with DM-ILD (642.3 +/- 498.4 U/mL) and healthy donors (162.4 +/- 54.01 U/mL). The KL-6 levels correlated positively with chest HRCT scores, serum lactate dehydrogenase, serum ferritin levels, and PFTs, but not with erythrocyte sedimentation rate. During follow-up, the post-treatment serum KL-6 levels significantly reduced in the remission/stable group, but increased in the acute exacerbation group. Higher levels of ferritin and KL-6 and HRCT scores were independently associated with poor prognosis. The 1-year survival rate was significantly lower in patients with high KL-6 level than in those with low KL-6 level. Conclusion The serum KL-6 levels may be a useful marker for predicting and monitoring ILD in Chinese patients with anti-MDA5(+) DM, especially amyopathic DM phenotype.