Incidence and Progression of Fibrotic Lung Disease in an At-Risk Cohort.
Incidence and Progression of Fibrotic Lung Disease in an At-Risk Cohort.
复制标题
高危人群中纤维化肺病的发病率和进展。
DOI：
10.1164/rccm.202206-1075oc
复制
发表时间：
2023
影响因子：
24.7
通讯作者：
Warren,R
中科院分区：
文献类型：
--
作者：
Steele,MarkP;Peljto,AnnaL;Mathai,SusanK;Humphries,Stephen;Bang,TamiJ;Oh,Andrea;Teague,Shawn;Cicchetti,Giuseppe;Sigakis,Christopher;Kropski,JonathanA;Loyd,JamesE;Blackwell,TimothyS;Brown,KevinK;Schwarz,MarvinI;Warren,R
理由：家族性间质性肺炎（FIP）患者的亲属发生肺纤维化和临床前肺纤维化（PrePF）的风险增加。目的：我们定义FIP家庭一级亲属中新发PrePF的发病率和进展及其与生存的关系。方法：这是一项对FIP家庭成员的队列研究，这些家庭成员最初通过健康问卷和胸部高分辨率计算机断层扫描（HRCT）进行筛查，大约4年后，再次进行评估。基线时共对493名FIP患者无症状一级亲属进行评估，296名（60%）原始受试者参加了后续评估。测量和主要结果：hrct之间的中位间隔为3.9年（四分位数范围为3.5-4.4年）。总共252名同意重复评价的受试者最初被确定在基线时不进行PrePF；16个国家发展了PrePF。pref年发病率的保守估计为1023 / 100000人年（95%置信区间为511 - 1831 / 100000人年）。在44名基线时患有PrePF的受试者中，38.4%的受试者出现呼吸困难加重，而未患有PrePF的受试者中这一比例为15.4% （P= 0.002）。HRCT常规间质性肺炎（P< 0.0002）和基线定量纤维化评分（P< 0.001）也与呼吸困难加重有关。初始筛查时的PrePF与生存率降低相关（P< 0.001）。结论：在这一高危人群中，PrePF的发生率至少比报道的散发性特发性肺纤维化（IPF）高100倍。虽然PrePF和IPF代表不同的实体，但我们的研究表明，PrePF和IPF一样，是进行性的，并与生存率降低有关。
Rationale:Relatives of patients with familial interstitial pneumonia (FIP) are at increased risk for pulmonary fibrosis and develop preclinical pulmonary fibrosis (PrePF).Objectives:We defined the incidence and progression of new-onset PrePF and its relationship to survival among first-degree relatives of families with FIP.Methods:This is a cohort study of family members with FIP who were initially screened with a health questionnaire and chest high-resolution computed tomography (HRCT) scan, and approximately 4 years later, the evaluation was repeated. A total of 493 asymptomatic first-degree relatives of patients with FIP were evaluated at baseline, and 296 (60%) of the original subjects participated in the subsequent evaluation.Measurements and Main Results:The median interval between HRCTs was 3.9 years (interquartile range, 3.5–4.4 yr). A total of 252 subjects who agreed to repeat evaluation were originally determined not to have PrePF at baseline; 16 developed PrePF. A conservative estimate of the annual incidence of PrePF is 1,023 per 100,000 person-years (95% confidence interval, 511–1,831 per 100,000 person-years). Of 44 subjects with PrePF at baseline, 38.4% subjects had worsening dyspnea compared with 15.4% of those without PrePF (P= 0.002). Usual interstitial pneumonia by HRCT (P< 0.0002) and baseline quantitative fibrosis score (P< 0.001) are also associated with worsening dyspnea. PrePF at the initial screen is associated with decreased survival (P< 0.001).Conclusions:The incidence of PrePF in this at-risk population is at least 100-fold higher than that reported for sporadic idiopathic pulmonary fibrosis (IPF). Although PrePF and IPF represent distinct entities, our study demonstrates that PrePF, like IPF, is progressive and associated with decreased survival.