Hypersensitivity Pneumonitis Radiologic Phenotypes Are Associated With Distinct Survival Time and Pulmonary Function Trajectory
Hypersensitivity Pneumonitis Radiologic Phenotypes Are Associated With Distinct Survival Time and Pulmonary Function Trajectory
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DOI：
10.1016/j.chest.2018.08.1076
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发表时间：
2019-04-01
期刊：
影响因子：
9.6
通讯作者：
Flaherty, Kevin R.
中科院分区：
文献类型：
--
作者：
Salisbury, Margaret L.;Gu, Tian;Flaherty, Kevin R.
背景技术背景：过敏性肺炎（HP）是一种间质性肺病，平均预后优于特发性肺纤维化（IPF）。我们通过放射学表型比较HP和IPF患者的生存时间和肺功能轨迹。方法：如果手术/经支气管肺活检、BAL和暴露史结果表明HP（n = 117）被诊断为HP。IPF（n = 152）经临床和组织病理学诊断。所有受试者均接受了基线高分辨率CT（HRCT）扫描和FVC %预测值。三位胸部放射科医生记录了放射学特征。生存时间是从HRCT扫描到死亡或肺移植。考克斯比例风险模型识别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tracks.RESULTS：受试者分组的临床诊断和三个相互排斥的放射学表型：蜂窝状，非蜂窝状纤维化（牵引性支气管扩张和网状）存在，和非纤维化。非纤维化HP患者的无事件中位生存期最长（> 14.73年），FVC %预测值改善（1.92%; 95% CI，0.49-3.35; P = 0.009）。HP伴非蜂窝状纤维化患者的生存期长于IPF（> 7.95 vs 5.20年），两组的FVC %预测值均显著下降。受试者HP和IPF与蜂窝生存率差（2.76和2.81年，分别）和FVC % predicted.CONCLUSIONS的显着下降：三个明确不同的，放射学定义的表型HP患者中确定。强调了在非蜂窝状纤维化患者中寻求特异性诊断（例如HP vs IPF）的重要性。当存在放射学蜂窝样改变时，由于预后一致较差，旨在确定诊断的侵入性诊断测试可能价值有限。
BACKGROUND: 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.METHODS: 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.RESULTS: 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.CONCLUSIONS: 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.