抗MDA5阳性皮肌炎和抗合成酶综合征中快速进展性间质性肺病的不同多变量危险因素。
Different Multivariable Risk Factors for Rapid Progressive Interstitial Lung Disease in Anti-MDA5 Positive Dermatomyositis and Anti-Synthetase Syndrome.
作者信息
Zuo Yu, Ye Lifang, Chen Fang, Shen Yawen, Lu Xin, Wang Guochun, Shu Xiaoming
机构信息
Department of Rheumatology, Key Laboratory of Myositis, China-Japan Friendship Hospital, Beijing, China.
Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.
出版信息
Front Immunol. 2022 Mar 7;13:845988. doi: 10.3389/fimmu.2022.845988. eCollection 2022.
BACKGROUND
Interstitial lung disease (ILD) is frequently observed in anti-melanoma differentiation-associated protein 5 (MDA5) antibody positive dermatomyositis (DM) and anti-synthetase syndrome (ASS), where they often develop a rapidly progressive ILD (RP-ILD) leading to poor prognosis.
OBJECTIVE
The aim of this study was to construct multivariable prediction risk factors for rapid progressive ILD (RP-ILD) in anti-MDA5 positive DM (MDA5DM) and ASS.
METHODS
333 idiopathic inflammatory myopathy (IIM) associated ILD patients were studied retrospectively. Risk factors for RP-ILD in MDA5DM and ASS patients were identified by univariate and multivariable logistic regression analysis. The mortality was assessed using Kaplan-Meier analysis.
RESULTS
RP-ILD was more prevalent in MDA5DM patients than ASS patients. MDA5DM patients with RP-ILD had significantly lower survival rates than those in ASS patients. The independent risk factors for RP-ILD in MDA5DM patients were fever (OR 3.67, 95% CI:1.79-7.52), lymphopenia (OR 2.14, 95% CI:1.01-4.53), especially decreased levels of CD3T cells (OR 2.56, 95% CI:1.17-5.61), decreased levels of CD3CD4 T cells (OR 2.80, 95% CI:1.37-5.73), CD3CD8T cells (OR 2.18, 95% CI:1.05-4.50), elevated CD5CD19 B cells (OR 3.17, 95% CI:1.41-7.13), elevated ALT (OR 2.36, 95% CI:1.15-4.81), high lactate dehydrogenase (LDH) (OR 3.08, 95% CI:1.52-6.27), hyper-ferritin (OR 4.97, 95% CI:1.97-12.50), elevated CEA (OR 2.28, 95% CI:1.13-4.59), and elevated CA153 (OR 3.31, 95% CI:1.50-7.27). While the independent risk factors for RP-ILD in ASS patients were elevated CEA (OR 5.25, 95% CI: 1.73-15.93), CA125 (OR 2.79, 95% CI: 1.10-7.11) and NSE (OR 4.86, 95% CI: 1.44-16.37). Importantly, serum ferritin>2200ng/ml predicted patient's death within half a year in MDA5DM patients with RP-ILD, but not in ASS patients.
CONCLUSIONS
There were significant different mortality and multivariable risk factors for RP-ILD in MDA5DM patients and ASS patients. Potential clinical benefits of using these different risk factors deserve assessment of severity and prognosis in IIM patients.
背景
间质性肺疾病（ILD）在抗黑色素瘤分化相关蛋白5（MDA5）抗体阳性皮肌炎（DM）和抗合成酶综合征（ASS）中经常出现，在这些疾病中，患者常发展为快速进展性ILD（RP-ILD），导致预后不良。
目的
本研究旨在构建抗MDA5阳性DM（MDA5DM）和ASS中快速进展性ILD（RP-ILD）的多变量预测危险因素。
方法
对333例特发性炎性肌病（IIM）相关ILD患者进行回顾性研究。通过单变量和多变量逻辑回归分析确定MDA5DM和ASS患者中RP-ILD的危险因素。使用Kaplan-Meier分析评估死亡率。
结果
RP-ILD在MDA5DM患者中比在ASS患者中更常见。患有RP-ILD的MDA5DM患者的生存率明显低于ASS患者。MDA5DM患者中RP-ILD的独立危险因素为发热（比值比3.67，95%置信区间：1.79-7.52）、淋巴细胞减少（比值比2.14，95%置信区间：1.01-4.53），尤其是CD3T细胞水平降低（比值比2.56，95%置信区间：1.17-5.61）、CD3CD4 T细胞水平降低（比值比2.80，95%置信区间：1.37-5.73）、CD3CD8T细胞水平降低（比值比2.18，95%置信区间：1.05-4.50）、CD5CD19 B细胞升高（比值比3.17，95%置信区间：1.41-7.13）、ALT升高（比值比2.36，95%置信区间：1.15-4.81）、高乳酸脱氢酶（LDH）（比值比3.08，95%置信区间：1.52-6.27）、高铁蛋白（比值比4.97，95%置信区间：1.97-12.50）、CEA升高（比值比2.28，95%置信区间：1.13-4.59）和CA153升高（比值比3.31，95%置信区间：1.50-7.27）。而ASS患者中RP-ILD的独立危险因素为CEA升高（比值比5.25，95%置信区间：1.73-15.93）、CA125升高（比值比2.79，95%置信区间：1.10-7.11）和NSE升高（比值比4.86，95%置信区间：1.44-16.37）。重要的是，血清铁蛋白>2200ng/ml可预测患有RP-ILD的MDA5DM患者在半年内死亡，但在ASS患者中则不然。
结论
MDA5DM患者和ASS患者中RP-ILD的死亡率和多变量危险因素存在显著差异。使用这些不同危险因素的潜在临床益处值得在IIM患者中评估疾病严重程度和预后。
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