与特发性肺纤维化相比，胸膜肺弹力纤维增生症的疾病过程和预后。
Disease course and prognosis of pleuroparenchymal fibroelastosis compared with idiopathic pulmonary fibrosis.
机构信息
Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
出版信息
Respir Med. 2020 Sep;171:106078. doi: 10.1016/j.rmed.2020.106078. Epub 2020 Jul 11.
BACKGROUND
Idiopathic pleuroparenchymal fibroelastosis (iPPFE) is a rare interstitial lung disease characterized by unique radiological and pathological findings. However, pathological evaluations are available only in a limited number of patients. Therefore, several clinical diagnostic criteria have been proposed. Nevertheless, the applicability of these criteria has not yet been validated. Moreover, the clinical course of iPPFE and its prognosis have not yet been completely elucidated.
METHODS
The present study assessed previously proposed clinical diagnostic criteria by comparing the clinical features between pathologically diagnosed iPPFE (p-iPPFE) and clinically diagnosed iPPFE (c-iPPFE). Subsequently, the clinical features of iPPFE were characterized and compared with those of idiopathic pulmonary fibrosis (IPF, n = 323).
RESULTS
Clinical characteristics of c-iPPFE (n = 27) and p-iPPFE (n = 35) were similar. No significant difference was observed in terms of prognosis between c-iPPFE and p-iPPFE. The number of patients with iPPFE (both c-iPPFE and p-iPPFE) who developed lung cancer was significantly lower than that of patients with IPF. However, acute exacerbation (AE) showed similar incidence in patients with iPPFE and IPF. Survival of patients with iPPFE was significantly worse than that of patients with IPF (5-year survival rate: 38.5% vs. 63.5%, p < 0.0001), and the most common cause of death was chronic respiratory failure (73.8%), followed by AE (14.3%). Male gender was the only poor prognostic factor of iPPFE.
CONCLUSION
The present study demonstrated efficiency of clinical diagnosis and also revealed clinically important characteristics of iPPFE that should be considered for management of iPPFE.
背景
特发性胸膜肺弹力纤维增生症（iPPFE）是一种罕见的间质性肺疾病，其影像学和病理学表现独特。然而，只有有限数量的患者可进行病理学评估。因此，已经提出了几种临床诊断标准。但是，这些标准的适用性尚未得到验证。此外，iPPFE 的临床病程及其预后尚未完全阐明。
方法
本研究通过比较经病理诊断的 iPPFE（p-iPPFE）和临床诊断的 iPPFE（c-iPPFE）患者的临床特征，评估了先前提出的临床诊断标准。随后，对 iPPFE 的临床特征进行了描述，并与特发性肺纤维化（IPF，n=323）进行了比较。
结果
c-iPPFE（n=27）和 p-iPPFE（n=35）的临床特征相似。c-iPPFE 和 p-iPPFE 患者的预后无显著差异。患有 iPPFE（包括 c-iPPFE 和 p-iPPFE）的患者发生肺癌的人数明显少于患有 IPF 的患者。然而，iPPFE 患者的急性加重（AE）发生率与 IPF 患者相似。iPPFE 患者的生存率明显差于 IPF 患者（5 年生存率：38.5% vs. 63.5%，p<0.0001），最常见的死亡原因是慢性呼吸衰竭（73.8%），其次是 AE（14.3%）。男性是 iPPFE 的唯一不良预后因素。
结论
本研究证明了临床诊断的有效性，并揭示了 iPPFE 的一些重要临床特征，这些特征应在 iPPFE 的管理中加以考虑。
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