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细胞角蛋白 19 片段与抗 MDA5 抗体阳性皮肌炎的间质性肺病严重程度和不良预后相关。

Cytokeratin 19 fragment is associated with severity and poor prognosis of interstitial lung disease in anti-MDA5 antibody-positive dermatomyositis.

机构信息

Department of Respiratory Medicine, The Affiliated Drum Tower Hospital of Nanjing University Medical School, No. 321 Zhongshan Road, Nanjing 210008, Jiangsu, P. R. China.

Department of Radiology, The Affiliated Drum Tower Hospital of Nanjing University Medical School, No. 321 Zhongshan Road, Nanjing 210008, Jiangsu, P. R. China.

出版信息

Rheumatology (Oxford). 2021 Aug 2;60(8):3913-3922. doi: 10.1093/rheumatology/keaa843.

DOI:10.1093/rheumatology/keaa843
PMID:33501503
Abstract

OBJECTIVES

In the present study, we aimed to assess the clinical significance of cytokeratin 19 fragment (CYFRA21-1) in patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive DM-interstitial lung disease (MDA5-DM-ILD).

METHODS

A total of 73 MDA5-DM-ILD patients were retrospectively analysed in this work. Their clinical characteristics, including clinical manifestations, laboratory findings, peripheral blood lymphocyte subsets and lung function, were compared between patients with acute/subacute interstitial pneumonia (A/SIP) and chronic interstitial pneumonia (CIP). The level of serum CYFRA21-1 was also compared between the above-mentioned two groups of patients, and its association with the clinical features and mortality of MDA5-DM-ILD was also evaluated.

RESULTS

Of the 73 MDA5-DM-ILD patients, 26 patients exhibited the A/SIP pattern. The level of serum CYFRA21-1 was higher in MDA5-DM patients with A/SIP compared with the CIP group (P = 0.009). Lower oxygenation index (OI), CD3+CD4+ T cell counts and percentage of CD3+CD4+ cells were also observed in MDA5-DM patients with A/SIP compared with the CIP group. Higher serum CYFRA21-1, lower OI, and lower zone consolidation were associated with a higher risk of A/SIP in MDA5-DM-ILD. In addition, 38 decedents with MDA5-DM-ILD exhibited a greater level of CYFRA21-1 compared with 35 survivors (P < 0.001). Furthermore, it was a prognostic factor and also associated with a higher mortality rate (log-rank test, P < 0.001).

CONCLUSIONS

CYFRA21-1 could be a useful serum indicator associated with occurrence of A/SIP in MDA5-DM-ILD. Moreover, it was associated with a poor survival in MDA5-DM-ILD patients.

摘要

目的

本研究旨在评估细胞角蛋白 19 片段(CYFRA21-1)在抗黑色素瘤分化相关基因 5(MDA5)抗体阳性皮肌炎-间质性肺病(MDA5-DM-ILD)患者中的临床意义。

方法

本研究回顾性分析了 73 例 MDA5-DM-ILD 患者。比较了急性/亚急性间质性肺炎(A/SIP)和慢性间质性肺炎(CIP)患者的临床特征,包括临床表现、实验室检查、外周血淋巴细胞亚群和肺功能。比较了两组患者血清 CYFRA21-1 水平,并评估其与 MDA5-DM-ILD 临床特征和死亡率的关系。

结果

73 例 MDA5-DM-ILD 患者中,26 例表现为 A/SIP 型。A/SIP 型 MDA5-DM 患者血清 CYFRA21-1 水平高于 CIP 组(P=0.009)。与 CIP 组相比,A/SIP 型 MDA5-DM 患者的氧合指数(OI)、CD3+CD4+T 细胞计数和 CD3+CD4+细胞百分比较低。MDA5-DM-ILD 患者中,血清 CYFRA21-1 水平较高、OI 较低、肺下野实变较多,发生 A/SIP 的风险较高。此外,38 例 MDA5-DM-ILD 死亡患者的 CYFRA21-1 水平高于 35 例存活患者(P<0.001)。此外,CYFRA21-1 是一个预后因素,与较高的死亡率相关(log-rank 检验,P<0.001)。

结论

CYFRA21-1 可能是 MDA5-DM-ILD 患者发生 A/SIP 的有用血清标志物。此外,它与 MDA5-DM-ILD 患者的不良预后相关。

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