Clinical significance of radiological pleuroparenchymal fibroelastosis pattern in interstitial lung disease patients registered for lung transplantation: a retrospective cohort study.
Clinical significance of radiological pleuroparenchymal fibroelastosis pattern in interstitial lung disease patients registered for lung transplantation: a retrospective cohort study.
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DOI：
10.1186/s12931-018-0860-6
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发表时间：
2018-08-30
影响因子：
5.8
通讯作者：
Date H
中科院分区：
文献类型：
--
作者：
Tanizawa K;Handa T;Kubo T;Chen-Yoshikawa TF;Aoyama A;Motoyama H;Hijiya K;Yoshizawa A;Oshima Y;Ikezoe K;Tokuda S;Nakatsuka Y;Murase Y;Nagai S;Muro S;Oga T;Chin K;Hirai T;Date H
放射学胸膜实质纤维弹性组织增生症（PPFE）病变的特征是胸膜增厚，高分辨率计算机断层扫描（HRCT）显示胸膜下纤维化的相关体征。本研究评价了放射学PPFE作为一种孤立发现或与其他间质性肺疾病（ILD）相关的临床意义，这些患者患有纤维化ILD并登记进行尸体肺移植（LT）。这项回顾性研究纳入了118例登记为LT的纤维化ILD患者。评估了HRCT上的放射学PPFE。评价放射学PPFE对临床特征和移植删失生存率的影响。在30/118例（25%）病例中观察到放射学PPFE：12例（10%）明确PPFE（PPFE集中在上叶，下叶受累不明显），18例（15%）一致PPFE（PPFE不集中在上叶，或PPFE伴其他部位共存疾病特征）。其中，12例患者在造血干细胞移植和/或化疗（LONIPC）后发生迟发性非感染性肺部并发症，9例为特发性PPFE，9例为其他纤维化ILD（特发性肺纤维化，IPF;其他特发性间质性肺炎，其他IIP;结缔组织病相关ILD，CTD-ILD和过敏性肺炎，HP）。放射学PPFE与既往气胸史、较低的体重指数、较低的预测用力肺活量（%FVC）百分比、较高的预测一氧化碳弥散量百分比、6分钟步行试验中较低的去饱和度和高碳酸血症相关。所有研究病例的中位生存时间为449天。37例（28%）接受了LT：31例为尸体，6例为活体供叶。93例未接受LT的患者中，66例（71%）死亡。校正年龄、性别、%FVC和6分钟步行距离< 250 m后，放射学PPFE与更好的生存率略有相关（风险比0.51 [0.25-1.05]，p = 0.07）。校正协变量后，特发性PPFE和LONIPC联合放射学PPFE的生存率高于无放射学PPFE的纤维化ILD（风险比0.38 [0.16-0.90]，p = 0.03），略高于其他纤维化ILD联合放射学PPFE（风险比0.20 [0.04-1.11]，p = 0.07）。在调整年龄、性别、%FVC和6分钟步行距离后，特发性PPFE和LONIPC伴放射学PPFE患者在LT等待名单上的生存率高于纤维化ILD伴放射学PPFE患者。本文的在线版本（10.1186/s12931-018-0860-6）包含补充材料，可供授权用户使用。
Radiological pleuroparenchymal fibroelastosis (PPFE) lesion is characterized by pleural thickening with associated signs of subpleural fibrosis on high-resolution computed tomography (HRCT). This study evaluated the clinical significance of radiological PPFE as an isolated finding or associated with other interstitial lung diseases (ILDs) in patients having fibrotic ILDs and registered for cadaveric lung transplantation (LT). This retrospective study included 118 fibrotic ILD patients registered for LT. Radiological PPFE on HRCT was assessed. The impact of radiological PPFE on clinical features and transplantation-censored survival were evaluated. Radiological PPFE was observed in 30/118 cases (25%): definite PPFE (PPFE concentrated in the upper lobes, with involvement of lower lobes being less marked) in 12 (10%) and consistent PPFE (PPFE not concentrated in the upper lobes, or PPFE with features of coexistent disease present elsewhere) in 18 (15%). Of these, 12 had late-onset non-infectious pulmonary complications after hematopoietic stem-cell transplantation and/or chemotherapy (LONIPCs), 9 idiopathic PPFE, and 9 other fibrotic ILDs (idiopathic pulmonary fibrosis, IPF; other idiopathic interstitial pneumonias, other IIPs; connective tissue disease-associated ILD, CTD-ILD, and hypersensitivity pneumonia, HP). Radiological PPFE was associated with previous history of pneumothorax, lower body mass index, lower percentage of predicted forced vital capacity (%FVC), higher percentage of predicted diffusion capacity of carbon monoxide, less desaturation on six-minute walk test, and hypercapnia. The median survival time of all study cases was 449 days. Thirty-seven (28%) received LTs: cadaveric in 31 and living-donor lobar in six. Of 93 patients who did not receive LT, 66 (71%) died. Radiological PPFE was marginally associated with better survival after adjustment for age, sex, %FVC, and six-minute walk distance < 250 m (hazard ratio 0.51 [0.25–1.05], p = 0.07). After adjustment for covariates, idiopathic PPFE and LONIPC with radiological PPFE was associated with better survival than fibrotic ILDs without radiological PPFE (hazard ratio 0.38 [0.16–0.90], p = 0.03), and marginally better survival than other fibrotic ILDs with radiological PPFE (hazard ratio, 0.20 [0.04–1.11], p = 0.07). idiopathic PPFE and LONIPC with radiological PPFE has better survival on the wait list for LT than fibrotic ILDs without radiological PPFE, after adjustment for age, sex, %FVC, and six-minute walk distance. The online version of this article (10.1186/s12931-018-0860-6) contains supplementary material, which is available to authorized users.
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