肝脏受累与抗黑色素瘤分化相关基因5抗体阳性皮肌炎患者快速进展性间质性肺病及死亡风险较高相关。
Liver Involvement is Associated with Higher Risk of Rapidly Progressive Interstitial Lung Disease and Mortality in Anti-Melanoma Differentiation-Associated Gene 5 Antibody- Positive Dermatomyositis.
作者信息
Yang Xin, Cui Xiaoguang, Yang Xiao, Ren Bincheng, Cheng Xiaojing, Zhao Xinrui, Liu Shanshan, Tian Tian, Zhao Hui, Qu Li, Li Xueyi
机构信息
Department of Rheumatology, Xi'an Jiaotong University Second Affiliated Hospital, Xi'an, People's Republic of China.
Scientific Research Center and Precision Medical Institute, Xi'an Jiaotong University Second Affiliated Hospital, Xi'an, People's Republic of China.
出版信息
J Inflamm Res. 2024 May 17;17:3101-3113. doi: 10.2147/JIR.S462721. eCollection 2024.
PURPOSE
This study aimed to assess liver involvement and investigate its correlation with rapidly progressive interstitial lung disease (RP-ILD) and mortality in anti-melanoma differentiation-associated gene 5 antibody-positive (anti-MDA5 positive) DM patients.
PATIENTS AND METHODS
This retrospective study included 159 patients diagnosed with anti-MDA5 positive DM or anti-synthetase syndrome (ASyS). Clinical features and laboratory findings were compared between patients with anti-MDA5 positive DM and patients with ASyS. In the anti-MDA5 positive DM cohort, clinical features and laboratory findings between patients with liver involvement and without liver involvement were further compared. The effects of liver involvement on the overall survival (OS) and development of RP-ILD were also analyzed using Kaplan-Meier method and Cox regression analysis.
RESULTS
Levels of serum aspartate aminotransferase (AST), alanine transaminase (ALT), γ-glutamyl transferase (γGT) and alkaline phosphatase (ALP) were all significantly higher in patients with anti-MDA5 positive DM than those in patients with ASyS. In our cohort of anti-MDA5 positive DM patents, 31 patients (34.4%) were complicated with liver involvement. Survival analysis revealed that serum ferritin >1030.0 ng/mL (p<0.001), ALT >103.0 U/l (p<0.001), AST >49.0 U/l (p<0.001), γGT >82.0 U/l (p<0.001), ALP >133.0 U/l (p<0.001), lactate dehydrogenase (LDH)>474.0 U/l (p<0.001), plasma albumin (ALB) <35.7 g/l (p<0.001) and direct bilirubin (DBIL) >2.80 μmol/l (p=0.002) predicted poor prognosis. The incidence of RP-ILD increased remarkably in patients with liver involvement compared to patients without liver involvement (58.1% vs 22.0%, p=0.001). Multivariate analysis revealed that elevated serum ALT level was an independent risk factor for mortality (HR 6.0, 95% CI 2.3, 16.2, p<0.001) and RP-ILD (HR 5.9, 95% CI 2.2, 15.9, p<0.001) in anti-MDA5 positive DM patents.
CONCLUSION
Liver involvement is common in patients with anti-MDA5 positive DM. Elevated serum ALT level was an independent risk factor for RP-ILD and mortality in patients with anti-MDA5 positive DM.
目的
本研究旨在评估抗黑色素瘤分化相关基因5抗体阳性（抗MDA5阳性）皮肌炎（DM）患者的肝脏受累情况，并研究其与快速进展性间质性肺病（RP-ILD）及死亡率的相关性。
患者与方法
本回顾性研究纳入了159例诊断为抗MDA5阳性DM或抗合成酶综合征（ASyS）的患者。比较了抗MDA5阳性DM患者与ASyS患者的临床特征和实验室检查结果。在抗MDA5阳性DM队列中，进一步比较了有肝脏受累和无肝脏受累患者的临床特征和实验室检查结果。还采用Kaplan-Meier法和Cox回归分析，分析了肝脏受累对总生存期（OS）和RP-ILD发生的影响。
结果
抗MDA5阳性DM患者的血清天冬氨酸转氨酶（AST）、丙氨酸转氨酶（ALT）、γ-谷氨酰转移酶（γGT）和碱性磷酸酶（ALP）水平均显著高于ASyS患者。在我们的抗MDA5阳性DM患者队列中，31例（34.4%）合并肝脏受累。生存分析显示，血清铁蛋白>1030.0 ng/mL（p<0.001）、ALT>103.0 U/l（p<0.001）、AST>49.0 U/l（p<0.001）、γGT>82.0 U/l（p<0.001）、ALP>133.0 U/l（p<0.001）、乳酸脱氢酶（LDH）>474.0 U/l（p<0.001）、血浆白蛋白（ALB）<35.7 g/l（p<0.001）和直接胆红素（DBIL）>2.80 μmol/l（p=0.002）提示预后不良。与无肝脏受累的患者相比（58.1%对22.0%，p=0.001），有肝脏受累的患者RP-ILD的发生率显著增加。多变量分析显示，血清ALT水平升高是抗MDA5阳性DM患者死亡（HR 6.0，95%CI 2.3，16.2，p<0.001）和RP-ILD（HR 5.9，95%CI 2.2，15.9，p<0.001）的独立危险因素。
结论
抗MDA5阳性DM患者中肝脏受累常见。血清ALT水平升高是抗MDA5阳性DM患者发生RP-ILD和死亡的独立危险因素。
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