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Abstract：
OBJECTIVE: To investigate the prognostic factors of patients with anti-melanoma differentiation-associated gene 5 (MDA5) positive clinically amyopathic dermatomyositis (CADM) and interstitial lung disease (ILD).
METHODS: A 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 χ2 test, Log-rank test, COX and logistic regression analysis.
RESULTS: In 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).
CONCLUSIONS: Patients 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.
METHODS: A 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 χ2 test, Log-rank test, COX and logistic regression analysis.
RESULTS: In 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).
CONCLUSIONS: Patients 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.
摘要：
目的：探讨抗黑色素瘤分化相关基因5(MDA5)阳性的临床肌病性皮肌炎(CADM)和间质性肺病(ILD)患者的预后因素。
方法：回顾性分析2014年12月至2022年12月华东地区10个分支的125例抗MDA5+CADM-ILD患者的临床资料。预后因素分析采用χ2检验,Log-ranktest,COX和logistic回归分析。
结果：在此队列中，125名抗MDA5+CADM-ILD患者表现出37.6%的快速进展性间质性肺病(RPILD)发病率,总死亡率为24.8%。一名患者失去了随访。诊断为RPILD后，在3个月内死亡的患者死亡率为53.2%，5.6%出现在存活3个月以上的人中。多因素分析显示,C反应蛋白(CRP)≥10mg/L(p=0.01)和含21(Ro52)(+)(p=0.003)的重组人三方基序与抗MDA5+CADM-ILD患者发生RPILD的风险较高相关;CRP≥10mg/L(p=0.018)和是否存在RPILD(p=0.003)是患者生存时间的影响因素。而关节炎是保护因素(p=0.016)。
结论：抗MDA5+CADM-ILD患者的死亡率更高，诊断为RPILD后的最初3个月被认为是预后不良的风险窗。CRP≥10mg/L的患者，Ro52(+)和RPILD可能与较短的生存时间有关，而患有关节炎的患者可能会出现相对温和的情况。
方法：回顾性分析2014年12月至2022年12月华东地区10个分支的125例抗MDA5+CADM-ILD患者的临床资料。预后因素分析采用χ2检验,Log-ranktest,COX和logistic回归分析。
结果：在此队列中，125名抗MDA5+CADM-ILD患者表现出37.6%的快速进展性间质性肺病(RPILD)发病率,总死亡率为24.8%。一名患者失去了随访。诊断为RPILD后，在3个月内死亡的患者死亡率为53.2%，5.6%出现在存活3个月以上的人中。多因素分析显示,C反应蛋白(CRP)≥10mg/L(p=0.01)和含21(Ro52)(+)(p=0.003)的重组人三方基序与抗MDA5+CADM-ILD患者发生RPILD的风险较高相关;CRP≥10mg/L(p=0.018)和是否存在RPILD(p=0.003)是患者生存时间的影响因素。而关节炎是保护因素(p=0.016)。
结论：抗MDA5+CADM-ILD患者的死亡率更高，诊断为RPILD后的最初3个月被认为是预后不良的风险窗。CRP≥10mg/L的患者，Ro52(+)和RPILD可能与较短的生存时间有关，而患有关节炎的患者可能会出现相对温和的情况。
