抗黑色素瘤分化抗原5抗体阳性皮肌炎合并间质性肺疾病亚型:临床特征与预后分析  

Subtypes of MDA5-positive dermatomyositis with interstitial lung disease:Clinical characteristics and prognostic analysis

作　　者：杨阚波 卢昕[1] 王国春[1] 陈芳[1] YANG Han-bo;LU Xin;WANG Guo-chun(Department of Rheumatology,China-Japan Friendship Hospital,Beijing 100029,China;不详)

机构地区：[1]中日友好医院风湿免疫内科,100029

出　　处：《中日友好医院学报》2024年第1期3-7,共5页Journal of China-Japan Friendship Hospital

基　　金：中央高校基本科研业务费专项资金(3332020074);中央高水平医院临床科研业务费(2022-NHLHCRF-YS-02)。

摘　　要：目的:探讨抗黑色素瘤分化抗原5(MDA5)抗体阳性皮肌炎(DM)并发间质性肺疾病(ILD)患者的临床特征及预后差异。方法:回顾性分析2012年1月—2021年7月收治的272例MDA5阳性DM-ILD患者。分为3组:组1为治疗前确诊为快速进展性ILD(RPILD),组2患者治疗后发生RPILD,组3为病情较轻的ILD。评估3组患者临床特征、治疗策略及糖皮质激素剂量等预后因素。结果:共纳入219例患者。组1患者病程短,发热、抗核抗体和抗Ro-52阳性率高,ESR、CRP水平高,CD3、CD4 T细胞计数低,与另2组患者差异有统计学意义(P<0.05)。组1患者治疗后4~8周死亡率高,组2患者12周后死亡率升高,组3死亡率最低,3组间死亡时间分布的差异有统计学意义(P<0.05)。Cox分析显示,发病年龄、乳酸脱氢酶水平、未用免疫抑制剂和初始激素用量是组1患者预后的独立危险因素,静脉注射免疫球蛋白是组2的保护因素(P<0.05)。组2多数患者肺部恶化时发现病原体。结论:MDA5阳性DM-ILD患者临床表现和预后存在显著异质性,RPILD患者并不能从大剂量激素治疗中获益。Objective:To explore the clinical characteristics and prognostic differences in patients with anti-melanoma differentiation antigen 5(MDA5)positive dermatomyositis(DM)complicated by interstitial lung disease(ILD).Methods:A retrospective analysis was conducted on 272 cases of MDA5-positive DM-ILD patients treated from 2012 to 2021.The patients weredivided into three groups:Group 1(pre-treatment rapidly progres⁃sive ILD,RPILD),Group 2(post-treatment RPILD),and Group 3(milder ILD).Clinical features,treatment strat⁃egies,and factors such as corticosteroid dosage were evaluated for prognosis.Results:The study included 219 patients.Group 1 had a shorter disease course,higher rates of fever,anti-nuclear antibodies,and anti-Ro-52 positivity,higher ESR and CRP levels,and lower CD3 and CD4 T-cell counts(P<0.05).Early mortality rates were higher in Group 1 after 4 and 8 weeks of treatment,while Group 2 showed increased mortality after 12 weeks,and Group 3 had the lowest mortality rate(P<0.05).Cox analysis revealed that age at onset,lactate de⁃hydrogenase levels,absence of immunosuppressants usage,and initial corticosteroid dosage were independent risk factors for prognosis in Group 1,while intravenous immunoglobulin was a protective factor for Group 2(P<0.05).Most Group 2 patients had detectable pathogens during pulmonary exacerbation.Conclusion:MDA5-pos⁃itive DM-ILD patients exhibit significant clinical heterogeneity and prognosis.RPILD patients do not benefit from high-dose corticosteroid treatment,underscoring a need for new therapeutic strategies.

关 键 词：抗黑色素瘤分化抗原5抗体 快速进展间质性肺疾病 临床异质性 皮肌炎 糖皮质激素 

分 类 号：R593.26[医药卫生—内科学]