Resende Ana C, Cordero Soraya, Mancuzo Eliane V, Storrer Karin M, Moreira Maria A, Baptista Fernanda M, Ferreira Rimarcs, Soares Maria Raquel, Pereira Carlos Alberto A
Department of Pulmonology, Federal University of São Paulo, São Paulo, BRA.
Department of Pulmonology, Federal University of Minas Gerais, Belo Horizonte, BRA.
Cureus. 2025 Mar 9;17(3):e80290. doi: 10.7759/cureus.80290. eCollection 2025 Mar.
This retrospective study aimed to determine the prevalence of progression in fibrotic interstitial lung disease (ILD) and the findings at diagnosis most associated with progression after two years of follow-up in a large Brazilian cohort.
This was a retrospective multicenter observational study in Brazil. Progression was defined after two years of follow-up. We excluded patients with an initial peripheral oxygen saturation (SpO2) of less than 88% or an initial forced vital capacity (FVC) of less than 45%. Diagnoses were made by multidisciplinary discussion. Patients with idiopathic pulmonary fibrosis were included for comparison. At least one of the following events was indicative of progressive ILD: (1) a relative decrease in FVC of 10% or more, (2) worsening dyspnea, (3) a greater extent of fibrotic findings on high-resolution computed tomography (HRCT), (4) initiation of oxygen, and (5) death attributed to ILD. Logistic regression analysis was used to identify risk factors for progressive fibrosis.
The mean age of patients was 61.7±12.3 years, and 69.5% had Velcro crackles. The mean FVC was 71.6±15.8%, and 26.1% showed honeycombing on HRCT. After two years of follow-up, 40.5% of patients (n=154) showed disease progression. Fibrotic hypersensitivity pneumonitis (FHP) was the most progressive disease (52%), and connective tissue disease-associated ILD (CTD-ILD) was the least progressive (25%). Multivariate analysis showed that a higher score for dyspnea, crackles, and SpO2 at rest ≤94% and ≤85% at the end of exercise were significant indicators of progression. Diffusing lung capacity for carbon monoxide (DLCO) was measured in 172 cases, with values <55% predicting a high odds ratio for progression (OR=4.03; 2.10-7.69).
In Brazil, FHP is the most progressive disease and CTD-ILD is the least progressive after two years of follow-up. The degree of dyspnea, crackles, SpO2 at rest and during exercise, and DLCO at baseline are associated with progressive disease.
这项回顾性研究旨在确定巴西一个大型队列中纤维化间质性肺疾病（ILD）的进展患病率以及随访两年后与进展最相关的诊断结果。
这是一项在巴西开展的回顾性多中心观察性研究。随访两年后定义疾病进展情况。我们排除了初始外周血氧饱和度（SpO2）低于88%或初始用力肺活量（FVC）低于45%的患者。诊断通过多学科讨论做出。纳入特发性肺纤维化患者作为对照。以下至少一项事件提示ILD进展：（1）FVC相对下降10%或更多，（2）呼吸困难加重，（3）高分辨率计算机断层扫描（HRCT）上纤维化表现范围扩大，（4）开始吸氧，（5）因ILD死亡。采用逻辑回归分析确定纤维化进展的危险因素。
患者的平均年龄为61.7±12.3岁，69.5%有捻发音。平均FVC为71.6±15.8%，26.1%在HRCT上显示蜂窝状改变。随访两年后，40.5%的患者（n = 154）出现疾病进展。纤维化性过敏性肺炎（FHP）是进展最明显的疾病（52%），结缔组织病相关ILD（CTD - ILD）进展最不明显（25%）。多变量分析显示，呼吸困难评分较高、有捻发音、静息时SpO2≤94%以及运动结束时SpO2≤85%是进展的显著指标。对172例患者进行了一氧化碳弥散量（DLCO）测量，DLCO值<55%预测进展的比值比很高（OR = 4.03；2.10 - 7.69）。
在巴西，随访两年后FHP是进展最明显的疾病，CTD - ILD进展最不明显。呼吸困难程度、捻发音、静息和运动时的SpO2以及基线时的DLCO与疾病进展相关。
