Rationale: Relatives of patients with familial pulmonary fibrosis (FPF) are at increased risk to develop FPF. Interstitial lung abnormalities (ILAs) are a radiologic biomarker of subclinical disease, but the implications of very mild abnormalities remain unclear. Objectives: To quantify the progression risk among FPF relatives with abnormalities below the threshold for ILAs as described by the Fleischner Society and to describe the characteristics of participants with new or progressive ILAs during observation. Methods: Asymptomatic FPF relatives undergo serial screening high-resolution chest computed tomography. For this analysis, early ILAs (no minimum threshold of lung involvement) were subclassified as mild (all interstitial abnormalities involve <5% of a lung zone) or moderate (any abnormality involves >5%). Identification of new or progressive ILAs on high-resolution chest computed tomography and the development of pulmonologist-diagnosed clinical FPF were defined as progression. Covariate-adjusted logistic regression identified progression-associated characteristics. Measurements and Main Results: From 2008 to 2023, 273 participants in follow-up procedures were 53.2 ± 9.4 years of age at enrollment, 95 (35%) were men, and 73 of 268 (27%) were ever-smokers. During a mean follow-up period of 6.2 ± 3.0 years, progression occurred among 31 of 211 (15%) of those with absence of ILAs at enrollment, 32 of 49 (65%) of those with mild ILAs, and 10 of 13 (77%) of those with moderate ILAs. Subjects with mild ILAs had 9.15 (95% confidence interval, 4.40-19.00; P < 0.0001) times and those with moderate ILAs had 17.14 (95% confidence interval, 4.42-66.49; P < 0.0001) times the odds of progression as subjects without ILAs. Conclusions: In persons at risk for FPF, minor interstitial abnormalities, including reticulation that is unilateral or involves <5% of a lung zone, frequently represent subclinical disease.

中文翻译：

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有家族性肺纤维化风险的人进行性早期间质性肺异常：一项前瞻性队列研究。


理由：家族性肺纤维化 （FPF） 患者的亲属患 FPF 的风险增加。间质性肺异常 （ILA） 是亚临床疾病的放射生物标志物，但非常轻微的异常的影响仍不清楚。目的：量化异常低于 Fleischner 协会描述的 ILA 阈值的 FPF 亲属的进展风险，并描述观察期间出现新发或进展性 ILA 的参与者的特征。方法： 无症状的 FPF 亲属接受系列筛查高分辨率胸部计算机断层扫描。对于此分析，早期 ILA （无肺部受累的最低阈值） 被细分为轻度 （所有间质异常涉及肺区的 <5%） 或中度 （任何异常涉及 >5%）。在高分辨率胸部计算机断层扫描中识别新的或进行性的 ILA 以及肺科医生诊断的临床 FPF 的发展被定义为进展。协变量调整的 logistic 回归确定了进展相关特征。测量和主要结果： 从 2008 年到 2023 年，273 名随访程序参与者的入组年龄为 53.2 ± 9.4 岁，95 名 （35%） 是男性，268 名中有 73 名 （27%） 是曾经吸烟的人。在 6.2 ± 3.0 年的平均随访期内，入组时没有 ILA 的患者中有 31 例 （15%），轻度 ILA 患者中 49 例中有 32 例 （65%），以及 13 例中度 ILA 患者中的 10 例 （77%）发生进展。轻度 ILA 受试者为 9.15 （95% 置信区间，4.40-19.00;P < 0.0001） 次，中度 ILA 患者为 17.14 （95% 置信区间，4.42-66.49;P < 0.0001） 是无 ILA 受试者进展几率的倍。 结论：在有 FPF 风险的人群中，轻微的间质异常，包括单侧或涉及肺区 <5% 的网状结构，通常代表亚临床疾病。