Prognostic analysis of MDA5-associated clinically amyopathic dermatomyositis with interstitial lung disease

被引:1
作者
Wang, Wen [1 ]
Sun, Xiang [2 ]
Xu, Yan [1 ]
Tan, Wenfeng [1 ,3 ]
Liu, Ye [4 ]
Zhou, Jun [1 ]
机构
[1] Nanjing Med Univ, Affiliated Suqian Peoples Hosp 1, Dept Rheumatol & Immunol, Suqian 223800, Peoples R China
[2] Jiangsu Prov Ctr Dis Control & Prevent, Expanded Program Immunizat, Nanjing, Peoples R China
[3] Nanjing Med Univ, Affiliated Hosp 1, Dept Rheumatol, Nanjing, Peoples R China
[4] Nanjing Med Univ, Affiliated Suqian Peoples Hosp 1, Dept Pharm, Suqian 223800, Peoples R China
关键词
anti-melanoma differentiation-associated gene 5; clinically amyopathic dermatomyositis; interstitial lung disease; prognosis; GENE; 5; ANTIBODY; ANTI-MDA5; GOTTRON SIGN; ULCERATION;
D O I
10.1002/iid3.1332
中图分类号
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号
100102 ;
摘要
ObjectiveTo investigate the prognostic factors of patients with anti-melanoma differentiation-associated gene 5 (MDA5) positive clinically amyopathic dermatomyositis (CADM) and interstitial lung disease (ILD).MethodsA retrospective analysis was conducted on clinical data of 125 patients with anti-MDA5 + CADM-ILD collected from 10 branches in eastern China between December 2014 and December 2022. Prognostic factors were analyzed using chi 2 test, Log-rank test, COX and logistic regression analysis.ResultsIn this cohort, 125 anti-MDA5 + CADM-ILD patients exhibited a rapidly progressive interstitial lung disease (RPILD) incidence of 37.6%, and an overall mortality rate of 24.8%. One patient was lost to follow-up. After diagnosis of RPILD, a mortality rate of 53.2% occurred in patients died within 3 months, and that of 5.6% appeared in those who survived for more than 3 months. Multiple factor analysis revealed that C-reactive protein (CRP) >= 10 mg/L (p = 0.01) and recombinant human tripartite motif containing 21 (Ro52) (+) (p = 0.003) were associated with a higher risk of RPILD in anti-MDA5 + CADM-ILD patients; CRP >= 10 mg/L (p = 0.018) and the presence of RPILD (p = 0.003) were identified as the factors influencing survival time in these patients, while arthritis was the protective factor (p = 0.016).ConclusionPatients with anti-MDA5 + CADM-ILD will have a higher mortality rate, and the initial 3 months after diagnosis of RPILD is considered the risk window for the dismal prognosis. Patients with CRP >= 10 mg/L, Ro52 (+) and RPILD may be related to a shorter survival time, while patients complicated with arthritis may present with relatively mild conditions.
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