抗黑色素瘤分化相关基因5抗体阳性皮肌炎的预后因素研究进展

张岑; 白灵; 李飞飞; 赵佩佩; 禚萃; 马文兰; 张思功

doi:10.16766/j.cnki.issn.1674-4152.003727

抗黑色素瘤分化相关基因5抗体阳性皮肌炎的预后因素研究进展

doi: 10.16766/j.cnki.issn.1674-4152.003727

1.
兰州大学第二临床医学院，甘肃兰州 730030
2.
青海大学附属医院风湿免疫科，青海西宁 810000
3.
兰州大学第二医院风湿免疫科，甘肃兰州 730030

基金项目:

国家自然科学基金项目 82060302

国家自然科学基金项目 82260325

兰州大学医学创新发展项目 lzuyxcx-2022-168

兰州大学第二医院萃英科技创新计划 CY2021-MS-A04

兰州大学第二医院萃英学子科研培育计划 CYXZ2021-47

详细信息

通讯作者:
张思功，E-mail：zhangsg@lzu.edu.cn

中图分类号: R593.26
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- 文章访问数: 27
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- 被引次数: 0
出版历程
- 收稿日期: 2024-01-23
- 网络出版日期: 2024-12-28

Advances in prognostic factors for anti-melanoma differentiation-associated gene 5 antibody positive dermatomyositis

1.
The Second Clinical Medical College of Lanzhou University, Lanzhou, Gansu 730030, China

摘要

摘要: 抗黑色素瘤分化相关基因5抗体阳性皮肌炎是特发性炎性肌病的一种特殊亚型，以皮肌炎的特征性皮损和间质性肺病为主要特点，易发生快速进展性间质性肺病，患者6个月死亡率极高，且不同个体之间的预后存在很大差异，因此早期预后评估对制定个体化诊疗方案具有重要意义。现有研究表明，高龄起病、男性、吸烟、发热、呼吸困难、胸腔积液、高滴度的抗黑色素瘤分化相关基因5抗体、抗黑色素瘤分化相关基因5抗体IgG1亚型阳性、抗Ro-52抗体阳性、血清C反应蛋白、乳酸脱氢酶、肌酸激酶等炎症指标升高、血清涎液化糖链抗原-6等肺损伤标记物升高、血清铁蛋白等巨噬细胞活化标志物升高、外周血淋巴细胞计数减少、氧合指数减低、用力肺活量占预计值的百分比减低是抗黑色素瘤分化相关基因5抗体阳性皮肌炎患者预后不良的危险因素；而病程较长、出现关节症状的患者往往预后较好。此外，有学者基于预后因素构建了风险预测模型，但现有的模型较为局限，全面、可靠的风险预测模型还有待进一步开发和验证。目前，抗黑色素瘤分化相关基因5抗体阳性皮肌炎尚缺乏有效的治疗，对于死亡高危患者，早期使用基于大剂量糖皮质激素的三联疗法、Janus激酶抑制剂、利妥昔单抗、血浆置换和预防性使用复方磺胺甲噁唑/甲氧苄啶等治疗有望改善患者预后。
- 抗黑色素瘤分化相关基因5抗体 /
- 皮肌炎 /
- 预后因素
Abstract: Anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis is a special subtype of idiopathic inflammatory myopathies, mainly characterized by dermatomyositis-specific skin lesions and interstitial lung disease. It is prone to rapidly progressive interstitial lung disease with a significantly high 6-month mortality rate. Besides, there is a great difference in prognosis among individuals. Therefore, early assessment of prognosis is crucial for the development of individualized diagnosis and therapeutic strategies. Available research suggests that several factors are associated with poor prognosis in anti-melanoma differentiation-related gene 5 antibody-positive dermatomyositis, including advanced age at onset, male gender, smoking, fever, dyspnea, pleural effusion, high titer of anti-melanoma differentiation-associated gene 5 antibodies, positive IgG1 subtype of anti-melanoma differentiation-associated gene 5 antibodies, positive anti-Ro-52 antibodies, elevated inflammatory markers (such as serum C-reactive protein, lactate dehydrogenase, and creatine kinase), elevated lung injury markers (such as serum Krebs von den Lungen-6), elevated markers of macrophage activation (such as serum ferritin), decreased peripheral blood lymphocyte counts, decreased oxygenation index, and a lower percentage of the predicted value for fprced vital capacity. The prognosis tends to be better for patients with a longer course of disease and joint symptoms. In addition, some scholars have constructed risk prediction models based on these prognostic factors. However, the existing models are relatively limited. More comprehensive and reliable risk prediction models need to be further developed and verified. Currently, effective treatments for anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis are lacking. For patients at high risk of death, early treatment with triple therapy, comprising high-dose glucocorticoids, Janus kinase inhibitors, rituximab, plasma exchange, and prophylactic use of cotrimoxazole/metronidazole, shows promise for improving prognosis.
- Anti-melanoma differentiation-associated gene 5 antibody /
- Dermatomyositis /
- Prognostic factors

HTML全文

表 1 MDA5⁺ DM患者死亡率和RP-ILD风险预测模型

Table 1. Risk prediction models for mortality and RP-ILD in patients with MDA5⁺DM

评分指标	意义	参考文献
以CRP≥0.8 mg/dL、KL-6≥1 000 U/mL为危险因素，根据危险因素个数评分：0~2分	MDA5⁺ DM患者的死亡风险预测：0分为低风险(＜15%)，1分为中风险(15%~50%)，2分为高风险(≥50%)	^[36]
病程≤4个月(5分)、发热(88分)、胸腔积液(21分)、总CT评分≥10分(22分)、AST≥200 U/L(100分)	MDA5⁺ DM-ILD患者的死亡风险预测：评分越高死亡风险越大	^[9]
年龄、向阳疹、发热、风险评分(包括淋巴细胞计数、CD3⁺CD4⁺T细胞计数、CYFRA21-1、氧合指数、抗Ro-52抗体)	MDA5⁺ DM-ILD患者的死亡风险预测：评分越高死亡风险越大	^[37]
胸部高分辨CT半定量评分：GGO总评分+2×实变评分	MDA5⁺ DM-ILD患者的死亡风险预测：评分＞18分的患者死亡风险高	^[38]
铁蛋白(≥636 ng/mL：2分)、乳酸脱氢酶(≥355 U/L：2分)、抗MDA5抗体(+：2分；++：3分；+++：4分)、胸部高分辨CT评分(≥133：3分)、RPILD (有：2分)	无肌病性皮肌炎相关ILD患者的死亡风险预测：0~4分为低风险，5~9分为中风险，10~13分为高风险	^[39]
发热、LDH＞300 IU/L、年龄＞50岁、NLR＞7为危险因素，根据危险因素个数评分：0~4分	MDA5⁺ DM患者发生RP-ILD的风险预测：评分越高RP-ILD发生的风险越大	^[5]
发热(5分)、甲周红斑(6分)、CRP升高(5分)、抗MDA5抗体阳性(8分)、抗Ro-52抗体阳性(6分)	DM患者发生RP-ILD的风险预测：0~9分为低风险，10~19分为中风险，20~30分为高风险	^[40]

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