Hoa Sabrina, Berger Claudie, Lahmek Nouha, Larché Maggie, Osman Mohammed, Choi May, Pope Janet, Thorne Carter, Hudson Marie
Centre Hospitalier de l'Université de Montréal, Montréal, Québec, Canada.
Research Institute of the McGill University Health Center, Montréal, Québec, Canada.
Arthritis Rheumatol. 2025 Apr;77(4):450-457. doi: 10.1002/art.43051. Epub 2025 Jan 7.
Interstitial lung disease (ILD) is a common and potentially lethal complication of systemic sclerosis (SSc). Screening by high-resolution computed tomography (HRCT) is recommended in all patients with risk factors, including early disease. Little is known on late presentations of ILD. This study aimed to characterize the incidence, risk factors, and outcomes of late-onset SSc-ILD.
Study participants enrolled in the Canadian Scleroderma Research Group cohort from 2004 to 2020 without prevalent ILD were included. Incidence and risk factors for ILD (on HRCT) were compared according to disease duration above (late) and below (earlier) seven years from the first non-Raynaud manifestation. Risk of ILD progression was compared using Kaplan-Meier and multivariable Cox models.
Overall, 199 (21%) of 969 patients developed incident ILD over a median of 2.4 (interquartile range 1.2-4.3) years. The incidence rate in late SSc (3.7/100 person-years) was lower than in earlier SSc (relative risk 0.68, 95% confidence interval [CI] 0.51-0.92). Risk factors for incident ILD included male sex, diffuse subtype, myositis, antitopoisomerase I autoantibodies, and higher C-reactive protein levels. Patients with late-onset ILD were also less frequently White and more frequently had arthritis and anti-RNA-polymerase III autoantibodies. Lung disease severity was similar between late- and earlier-onset SSc-ILD (forced vital capacity 88% and 87%, diffusion capacity of the lungs for carbon monoxide 64% and 62%, respectively). Progression rates were also similar between late- and earlier-onset SSc-ILD (log rank P = 0.8, hazard ratio 1.11, 95% CI 0.58-2.10).
ILD can present in late SSc. Risk factors and progression rates overlapped with earlier-onset SSc-ILD. Surveillance for ILD should continue in longstanding SSc. Frequency and modality of monitoring remain to be defined.
间质性肺疾病（ILD）是系统性硬化症（SSc）常见且可能致命的并发症。建议对所有有风险因素的患者进行高分辨率计算机断层扫描（HRCT）筛查，包括疾病早期患者。关于ILD的晚期表现知之甚少。本研究旨在描述迟发性SSc-ILD的发病率、风险因素和结局。
纳入2004年至2020年参加加拿大硬皮病研究组队列且无ILD病史的研究参与者。根据首次非雷诺现象出现后七年以上（晚期）和七年以下（早期）的疾病持续时间，比较ILD（基于HRCT）的发病率和风险因素。使用Kaplan-Meier和多变量Cox模型比较ILD进展风险。
总体而言，969例患者中有199例（21%）在中位时间2.4年（四分位间距1.2 - 4.3年）内发生了新发ILD。晚期SSc的发病率（3.7/100人年）低于早期SSc（相对风险0.68，95%置信区间[CI]0.51 - 0.92）。新发ILD的风险因素包括男性、弥漫性亚型、肌炎、抗拓扑异构酶I自身抗体以及较高的C反应蛋白水平。迟发性ILD患者中白人较少见，关节炎和抗RNA聚合酶III自身抗体更为常见。晚期和早期SSc-ILD的肺部疾病严重程度相似（用力肺活量分别为88%和87%，肺一氧化碳弥散量分别为64%和62%）。晚期和早期SSc-ILD的进展率也相似（对数秩检验P = 0.8，风险比1.11，95% CI 0.58 - 2.10）。
ILD可出现在晚期SSc中。风险因素和进展率与早期SSc-ILD重叠。对于病程较长的SSc患者，应继续监测ILD。监测的频率和方式仍有待确定。
