Division of Pulmonary and Critical Care Medicine, University of Michigan, Ann Arbor, MI.
Department of Biostatistics, University of Michigan, Ann Arbor, MI.
Chest. 2019 Apr;155(4):699-711. doi: 10.1016/j.chest.2018.08.1076. Epub 2018 Sep 19.
Hypersensitivity pneumonitis (HP) is an interstitial lung disease with a better prognosis, on average, than idiopathic pulmonary fibrosis (IPF). We compare survival time and pulmonary function trajectory in patients with HP and IPF by radiologic phenotype.
HP (n = 117) was diagnosed if surgical/transbronchial lung biopsy, BAL, and exposure history results suggested this diagnosis. IPF (n = 152) was clinically and histopathologically diagnosed. All participants had a baseline high-resolution CT (HRCT) scan and FVC % predicted. Three thoracic radiologists documented radiologic features. Survival time is from HRCT scan to death or lung transplant. Cox proportional hazards models identify variables associated with survival time. Linear mixed models compare post-HRCT scan FVC % predicted trajectories.
Subjects were grouped by clinical diagnosis and three mutually exclusive radiologic phenotypes: honeycomb present, non-honeycomb fibrosis (traction bronchiectasis and reticulation) present, and nonfibrotic. Nonfibrotic HP had the longest event-free median survival (> 14.73 years) and improving FVC % predicted (1.92%; 95% CI, 0.49-3.35; P = .009). HP with non-honeycomb fibrosis had longer survival than IPF (> 7.95 vs 5.20 years), and both groups experienced a significant decline in FVC % predicted. Subjects with HP and IPF with honeycombing had poor survival (2.76 and 2.81 years, respectively) and significant decline in FVC % predicted.
Three prognostically distinct, radiologically defined phenotypes are identified among patients with HP. The importance of pursuing a specific diagnosis (eg, HP vs IPF) among patients with non-honeycomb fibrosis is highlighted. When radiologic honeycombing is present, invasive diagnostic testing directed at determining the diagnosis may be of limited value given a uniformly poor prognosis.
过敏性肺炎 (HP) 是一种间质性肺疾病，其平均预后好于特发性肺纤维化 (IPF)。我们通过影像学表型比较 HP 和 IPF 患者的生存时间和肺功能轨迹。
如果手术/经支气管肺活检、BAL 和暴露史结果提示诊断为 HP，则诊断为 HP (n=117)。IPF (n=152) 为临床和组织病理学诊断。所有参与者均进行基线高分辨率 CT (HRCT) 扫描和 FVC % 预测。三名胸部放射科医生记录放射学特征。生存时间为从 HRCT 扫描到死亡或肺移植。Cox 比例风险模型确定与生存时间相关的变量。线性混合模型比较 HRCT 扫描后 FVC % 预测轨迹。
根据临床诊断和三种互斥的影像学表型对受试者进行分组：存在蜂窝、存在非蜂窝纤维化（牵引性支气管扩张和网状）和非纤维化。非纤维化 HP 无事件生存的中位生存期最长 (>14.73 年)，FVC % 预测值改善 (1.92%；95%CI，0.49-3.35；P=.009)。具有非蜂窝纤维化的 HP 比 IPF 的生存期更长 (>7.95 比 5.20 年)，两组的 FVC % 预测值均显著下降。具有蜂窝的 HP 和 IPF 患者的生存情况较差 (分别为 2.76 和 2.81 年)，且 FVC % 预测值均显著下降。
在 HP 患者中确定了三种具有不同预后的影像学定义表型。在非蜂窝纤维化患者中，明确特定诊断 (如 HP 与 IPF) 的重要性得到强调。当存在影像学蜂窝时，针对确定诊断的侵入性诊断测试的价值可能有限，因为预后普遍较差。
