Department of Academic Rheumatology, King's College London, London, UK.
Deparment of Rheumatology, King's College Hospital, London, UK.
Rheumatology (Oxford). 2024 Nov 1;63(11):3080-3090. doi: 10.1093/rheumatology/kead638.
The impact of autoantibody profiles on the prognosis for idiopathic inflammatory myositis-associated interstitial lung disease (IIM-ILD) and myositis spectrum ILD with myositis-specific antibodies (MSAs) remains unclear. This retrospective cohort study examined whether serological profiles were associated with mortality or longitudinal lung function change.
The baseline clinical/demographic characteristics and follow-up lung function data of consecutive adult patients with IIM-ILD or interstitial pneumonia with autoimmune features (IPAF) positive for MSAs (IPAF-MSA) were extracted from three hospitals. Univariate and multivariate Cox proportional hazards analyses were used to compare mortality between groups of patients with different autoantibodies. Regression models were used to analyse their lung function trends.
Of the 430 included patients, 81% met the IIM criteria, and the remaining 19% were diagnosed with IPAF-MSA. On univariate analysis, the risk factors associated with mortality included higher age, Charlson Comorbidity Index, and CRP; and lower BMI, baseline TLCO% and FEV1%. Compared with anti-MDA5 negativity, anti-MDA5 positivity (MDA5+) was associated with higher mortality in the first 3 months [hazard ratio (HR) 65.2, 95% CI 14.1, 302.0], while no significant difference was seen thereafter (HR 0.55, 95% CI 0.14, 2.28). On multivariate analysis, combined anti-synthetase antibodies were associated with a reduced risk of mortality (HR 0.63), although individually, mortality was reduced in patients with anti-Jo1+ (HR 0.61, 95% CI 0.4-0.87) and increased in patients with anti-PL7+ (HR 2.07, 95% CI 1.44-2.99). Anti-MDA5+ was associated with slow improvement in %FVC over the first 3 years, while anti-PL7+ was linked with a slow decline from 12 months onwards.
Among the autoantibody profiles in myositis spectrum disorders, anti-MDA5+ and anti-PL7+ conferred higher mortality risks in patients with IIM-ILD. Survivors of an early peak of mortality in anti-MDA5+ disease appeared to have a favourable prognosis.
自身抗体谱对特发性炎性肌病相关间质性肺病（IIM-ILD）和肌炎特异性抗体（MSA）阳性的肌炎谱间质性肺病（MSA-ILD）的预后影响尚不清楚。本回顾性队列研究旨在探讨血清学特征是否与死亡率或纵向肺功能变化相关。
从三所医院提取连续的成年 IIM-ILD 或 MSA 阳性的自身免疫性特征性间质性肺炎（IPAF-MSA）患者的基线临床/人口统计学特征和随访肺功能数据。采用单因素和多因素 Cox 比例风险分析比较不同自身抗体患者组的死亡率。采用回归模型分析其肺功能趋势。
在纳入的 430 例患者中，81%符合 IIM 标准，其余 19%被诊断为 IPAF-MSA。单因素分析显示，与死亡率相关的危险因素包括年龄较大、Charlson 合并症指数和 CRP 较高；BMI、基线 TLCO%和 FEV1%较低。与抗 MDA5 阴性相比，抗 MDA5 阳性（MDA5+）患者在头 3 个月的死亡率更高[风险比（HR）65.2，95%置信区间（CI）14.1，302.0]，但此后无显著差异（HR 0.55，95%CI 0.14，2.28）。多因素分析显示，同时存在抗合成酶抗体与死亡率降低相关（HR 0.63），尽管单独来看，抗 Jo1+患者的死亡率降低（HR 0.61，95%CI 0.4-0.87），而抗 PL7+患者的死亡率增加（HR 2.07，95%CI 1.44-2.99）。抗 MDA5+与前 3 年 %FVC 的改善缓慢相关，而抗 PL7+与 12 个月后开始的缓慢下降相关。
在肌炎谱疾病的自身抗体谱中，抗 MDA5+和抗 PL7+在 IIM-ILD 患者中具有更高的死亡风险。抗 MDA5+疾病早期死亡率峰值的存活者似乎预后良好。
