Clinical characteristics and treatment evaluation of anti-melanoma differentiation-associated protein-5 antibody-positive dermatomyositis patients with fatal outcomes: a retrospective analysis
10.3760/cma.j.cn141217-20240514-00159
- VernacularTitle:抗黑色素瘤分化相关基因5抗体阳性皮肌炎死亡患者临床特征及治疗评估回顾性分析
- Author:
Xiaoguang CUI
1
;
Xin YANG
1
;
Bincheng REN
1
;
Xiaojing CHENG
1
;
Shanshan LIU
1
;
Xinrui ZHAO
1
;
Tian TIAN
1
;
Hui ZHAO
1
;
Xueyi LI
1
Author Information
1. 西安交通大学第二附属医院风湿免疫科，西安 710000
- Publication Type:Journal Article
- Keywords:
Dermatomyositis;
Lung diseases, interstitial;
Anti-melanoma differentiation-associated protein-5 antibody
- From:
Chinese Journal of Rheumatology
2025;29(3):204-208
- CountryChina
- Language:Chinese
-
Abstract:
Objective:This study aims to provide insights into the clinical features of anti-melanoma differentiation-associated protein-5(MDA5)-positive dermatomyositis (MDA5-DM) patients with fatal outcomes, leveraging pathogenic microbiota metagenomic analysis, to guide the clinical assessment and treatment choices.Methods:From January 2020 to August 2023, deceased patients diagnosed with MDA5-DM were identified at the Department of Rheumatology and Immunology, the Second Affiliated Hospital of Xi ′an Jiaotong University. Clinical data were retrospectively collected and analyzed using Mann Whitney U test and Fisher ′s exact test to summarize risk factors and treatment assessment for MDA5-DM patients with fatal outcomes. Results:①The proportion of male patients was higher than females among MDA5-DM patients with fatal outcomes, which differed from the incidence pattern, possibly associated with smoking and gender proportions (6/11 vs. 0/7, P=0.037). ②94%(17/18) patients presented initially with elevated ferritin levels [(1 350±942)ng/ml] and CRP [(47±36)mg/L]. ③All patients (18/18) exhibited early involvement of the upper lung lobes, including multiple nodules in 9/18, ground-glass opacities in 5/18, and solitary nodules in 4/18. ④Metagenomic next-generation sequencing (mNGS) of bronchoalveolar lavage fluid was negative in 4/16 cases, with cytomegalovirus and pneumocystis jirovecii being the most commonly detected pathogens in 5/16 cases each. ⑤89%(16/18) of patients continued to have lymphocyte counts persistently <0.5×10 9/L irrespective of treatment. Conclusion:Smoking may have adverse effects on male MDA5 patients. Early involvement of the upper lobe of the lungs is more common in MDA5 antibody positive deaths, and persistent lymphocyte depletion is an important factor in poor response. Enhancing mNGS analysis of bronchoalveolar lavage fluid and vigilance towards cytomegalovirusand Pneumocystis jirovecii could provide valuable clinical guidance.
