Familial pulmonary fibrosis: Clinical and radiological characteristics and progression analysis in different high resolution-CT patterns
Familial pulmonary fibrosis: Clinical and radiological characteristics and progression analysis in different high resolution-CT patterns
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DOI：
10.1016/j.rmed.2017.03.020
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发表时间：
2017-05-01
影响因子：
4.3
通讯作者：
Rottoli, Paola
中科院分区：
文献类型：
--
作者：
Bennett, David;Mazzei, Maria Antonietta;Rottoli, Paola
背景：家族性肺纤维化(FPF)被定义为一种特发性弥漫性肺病，可累及同一原始生物家族的两个或多个成员。本研究的目的是从疾病进展和存活率的角度对临床、功能和放射学特征做出贡献。方法：根据2011年IPF指南的HRCT分类，对46例FPF患者的临床、功能和放射学资料进行了回顾性收集和分析。结果：本研究共纳入30个家系，女性22例，男性24例，年龄58.5+/-9.7岁。放射学分析显示54.3%的患者在HRCT上存在UIP模式，21.8%的患者为POSS-UIP，23.9%的患者为INCON-UIP。INCON-UIP患者较年轻，且多为女性。肺功能检查显示UIP和INCON-UIP模式的患者存在限制性呼吸缺陷，而POSS-UIP患者的肺容量正常，仅有轻微的DLCO减少。BAL成分显示INCON-UIP患者淋巴细胞百分率升高。呼吸功能1年随访显示，只有UIP患者的情况明显恶化。HRCT仅显示从POSS-UIP到UIP的进展(18%的患者)。虽然POSS-UIP患者的预后较好，但3组HRCT患者的中位生存期无统计学差异。结论：FPF是一种复杂的疾病，预后较差。本研究首先分析了FPF患者的功能和放射学随访资料，发现其可能表现为几种不同进展速度的HRCT表现，其中UIP和UIP可能被认为是同一疾病的不同阶段，而不一致的UIP患者可能代表不同的临床和放射学状况。(C)2017爱思唯尔有限公司。保留所有权利。
Background: Familial pulmonary fibrosis (FPF) is defined as an idiopathic diffuse parenchymal lung disease affecting two or more members of the same primary biological family. The aim of the present study was to contribute to the clinical, functional and radiological characterisation of FPF with particular regards to disease progression and survival.Methods: Baseline clinical, functional and radiological data of a FPF population (n = 46 patients) were retrospectively collected and analysed according to the 2011 IPF guidelines HRCT classification. A PFT follow-up after 1-year and survival analysis was conducted among to different HRCT patterns.Results: 22 female and 24 male patients (age at diagnosis 58.5 +/- 9.7 years-old), belonging to 30 families, were included in this study. Radiological analysis demonstrated the presence of a UIP pattern at HRCT in 54.3% of patients, Poss-UIP in 21.8% and Incon-UIP in 23.9%. Incon-UIP patients were younger and more frequently female. Pulmonary function tests showed a restrictive ventilatory defect in patients with UIP and Incon-UIP patterns, while Poss-UIP patients had normal volumes with only a mild reduction of DLCO. BAL composition revealed increased lymphocytes percentage in Incon-UIP patients. Respiratory functional 1-year follow-up showed a significant worsening in UIP patients only. HRCT pattern progression was only demonstrated from Poss-UIP to UIP (18% of patients). Median survival was not statistically different among the 3 HRCT groups, although Poss-UIP patients presented a better outcome.Conclusions: FPF has been confirmed to be a complex condition with poor prognosis. The present study firstly analysed functional and radiological follow-up data of patients with FPF, showing that it may manifests with several HRCT patterns with different rates of progression, in which Possible UIP and UIP could be considered phases of the same disease and Inconsistent UIP patients may represent a different clinical and radiological condition. (C) 2017 Elsevier Ltd. All rights reserved.