Immune Inflammation and Disease Progression in Idiopathic Pulmonary Fibrosis.
Immune Inflammation and Disease Progression in Idiopathic Pulmonary Fibrosis.
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DOI：
10.1371/journal.pone.0154516
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发表时间：
2016
期刊：
影响因子：
3.7
通讯作者：
Saetta M
中科院分区：
文献类型：
--
作者：
Balestro E;Calabrese F;Turato G;Lunardi F;Bazzan E;Marulli G;Biondini D;Rossi E;Sanduzzi A;Rea F;Rigobello C;Gregori D;Baraldo S;Spagnolo P;Cosio MG;Saetta M
特发性肺纤维化（IPF）的临床病程具有高度异质性，一些患者进展缓慢，另一些患者临床和功能下降加速。本研究旨在临床表征IPF进展类型，并研究可能解释观察到的疾病行为差异的病理学基础。分析了73例IPF患者的临床和功能数据，这些患者作为肺移植的候选人长期随访。用力肺活量（FVC）变化/年（<或≥10%预测值）用于定义“缓慢”或“快速”疾病进展。在73例接受肺移植的患者中，有41例的移植肺病理学异常被量化。诊断时，缓慢进展者（n = 48）的症状持续时间较长，FVC低于快速进展者（n = 25）。11例缓慢进展者和3例快速进展者在随访期间发生急性加重（AE）。定量肺病理学显示，快速进展者中存在重度先天性和适应性炎症浸润，与缓慢进展者相比显著增加，与发生AE的患者中观察到的结果相似。炎症程度与FVC年下降相关（r = 0.52，p = 0.005）。总之，先天性和适应性炎症似乎是IPF患者肺部的一个突出特征，可能有助于确定疾病进展的速率。
The clinical course in idiopathic pulmonary fibrosis (IPF) is highly heterogeneous, with some patients having a slow progression and others an accelerated clinical and functional decline. This study aims to clinically characterize the type of progression in IPF and to investigate the pathological basis that might account for the observed differences in disease behavior. Clinical and functional data were analyzed in 73 IPF patients, followed long-time as candidates for lung transplantation. The forced vital capacity (FVC) change/year (< or ≥10% predicted) was used to define “slow” or “rapid” disease progression. Pathological abnormalities were quantified in the explanted lung of 41 out of 73 patients undergoing lung transplantation. At diagnosis, slow progressors (n = 48) showed longer duration of symptoms and lower FVC than rapid progressors (n = 25). Eleven slow and 3 rapid progressors developed an acute exacerbation (AE) during follow-up. Quantitative lung pathology showed a severe innate and adaptive inflammatory infiltrate in rapid progressors, markedly increased compared to slow progressors and similar to that observed in patients experiencing AE. The extent of inflammation was correlated with the yearly FVC decline (r = 0.52, p = 0.005). In conclusion an innate and adaptive inflammation appears to be a prominent feature in the lung of patients with IPF and could contribute to determining of the rate of disease progression.