Tzilas Vasilios, Tzouvelekis Argyris, Bouros Evangelos, Karampitsakos Theodoros, Ntassiou Maria, Avdoula Eleni, Trachalaki Athena, Antoniou Katerina, Raghu Ganesh, Bouros Demosthenes
First Academic Dept of Pneumonology, Interstitial Lung Diseases Unit, Hospital for Diseases of the Chest, "Sotiria", Medical School, National and Kapodistrian University of Athens, Athens, Greece.
These authors contributed equally.
ERJ Open Res. 2020 Nov 2;6(4). doi: 10.1183/23120541.00152-2020. eCollection 2020 Oct.
Fibrotic hypersensitivity pneumonitis (f-HP) can exhibit a progressive course similar to idiopathic pulmonary fibrosis (IPF). The lack of diagnostic guidelines and randomised controlled trials in this population represent a significant unmet need.
To describe our clinical experience with antifibrotics in patients with f-HP.
Retrospective study of 30 patients diagnosed with f-HP upon re-evaluation within a multidisciplinary team discussion of 295 consecutive patients (January 2012 to December 2017) who had been diagnosed initially with IPF at outside facilities and were referred to our centres.
Pirfenidone was initially administered to 14 (46.7%) patients and nintedanib to 16 (53.3%) patients. There were 26 (86.7%) males, with mean±sd age 70.2±8.4 years. The annual rate of decline in forced vital capacity (FVC) % predicted over the 3-year treatment period adjusted for baseline FVC % pred measurement was 4.2% (95% CI 1.9-6.6%, p=0.001) and 7.5% (95% CI 3.3-11.7%; p=0.001) in imputation analysis. The annual rate of decline in diffusing capacity of the lung for carbon monoxide ( ) % predicted throughout the 3-year treatment period adjusted for baseline % pred was 5.7% (95% CI 3.1-8.4%, p<0.001) and 5.8% (95% CI 3.4-8.1%, p<0.001) in imputation analysis. The nature of adverse events was related to the type of antifibrotic agent administered.
In patients with f-HP receiving antifibrotics there is a statistically significant annual decline in FVC % pred and % pred over a period of 3 years. Prospective randomised trials exceeding 1 year are warranted to determine the long-term efficacy of antifibrotics.
纤维化性过敏性肺炎（f-HP）可呈现与特发性肺纤维化（IPF）相似的进行性病程。该人群缺乏诊断指南和随机对照试验，这是一个重大的未满足需求。
描述我们在f-HP患者中使用抗纤维化药物的临床经验。
对295例连续患者（2012年1月至2017年12月）进行回顾性研究，这些患者最初在外院被诊断为IPF，后转诊至我们中心，在多学科团队讨论中经重新评估确诊为f-HP的患者有30例。
14例（46.7%）患者最初使用吡非尼酮，16例（53.3%）患者使用尼达尼布。男性26例（86.7%），平均年龄70.2±8.4岁。在插补分析中，经基线用力肺活量（FVC）预计值百分比测量调整后的3年治疗期内，FVC预计值百分比的年下降率为4.2%（95%置信区间1.9-6.6%，p=0.001）和7.5%（95%置信区间3.3-11.7%；p=0.001）。经基线一氧化碳弥散量（）预计值百分比调整后的3年治疗期内，一氧化碳弥散量预计值百分比的年下降率在插补分析中为5.7%（95%置信区间3.1-8.4%，p<0.001）和5.8%（95%置信区间3.4-8.1%，p<0.001）。不良事件的性质与所使用抗纤维化药物的类型有关。
在接受抗纤维化药物治疗的f-HP患者中，3年内FVC预计值百分比和预计值百分比每年有统计学意义的下降。有必要进行超过1年的前瞻性随机试验以确定抗纤维化药物的长期疗效。
