Comparison of Clinical Characteristics and Outcomes between Idiopathic and Secondary Pleuroparenchymal Fibroelastosis.
Comparison of Clinical Characteristics and Outcomes between Idiopathic and Secondary Pleuroparenchymal Fibroelastosis.
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DOI：
10.3390/jcm10040846
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发表时间：
2021-02-18
影响因子：
3.9
通讯作者：
Ogura T
中科院分区：
文献类型：
--
作者：
Oda T;Sekine A;Tabata E;Iwasawa T;Takemura T;Ogura T
背景资料：胸膜实质弹力纤维增生症（PPFE）是一种独特的临床、影像学和组织病理学实体，最近已报道了几种潜在的病因。然而，还没有对继发性PPFE进行全面的研究。目的：评估继发性和特发性PPFE的临床特征、结局和预后因素。方法：我们回顾性分析了1999年1月至2018年12月期间连续PPFE患者的病历。我们确定了132例原发性PPFE患者和32例继发性PPFE患者。结果如下：继发性PPFE患者（38.5%）中与下叶常见间质性肺炎（UIP）模式不同的间质性肺病（ILD）模式的发生率高于特发性PPFE患者（61.5%，p = 0.02）。特发性和继发性PPFE组在实验室数据、呼吸系统并发症和生存期方面无显著差异（中位数：5.0年vs. 4.1年，p = 0.95）。在多变量分析中，UIP模式的存在与原发性PPFE患者的死亡率增加独立相关，但与继发性PPFE患者无关。结论：特发性和继发性PPFE患者UIP型ILD的发生率和预后影响不同。对于肺一氧化碳弥散量（DLCO）低的继发性PPFE患者，无论下叶ILD类型如何，均应考虑肺移植。
Background: Pleuroparenchymal fibroelastosis (PPFE) is a unique clinical, radiologic, and histopathologic entity for which several potential etiologies have been reported recently. However, there has been no comprehensive study of secondary PPFE. Objective: Assessment of the clinical characteristics, outcomes, and prognostic factors of secondary and idiopathic PPFE. Methods: We retrospectively reviewed the medical records of consecutive PPFE patients between January 1999 and December 2018. We identified 132 idiopathic PPFE patients and 32 secondary PPFE patients. Results: The incidence of interstitial lung disease (ILD) pattern different from the usual interstitial pneumonia (UIP) pattern in the lower lobes was higher in secondary PPFE patients (38.5%) than in idiopathic PPFE patients (61.5%, p = 0.02). The idiopathic and secondary PPFE groups did not differ significantly in terms of laboratory data, respiratory complications, and survival (median: 5.0 years vs. 4.1 years, p = 0.95). The presence of UIP pattern was independently associated with increased mortality in multivariate analyses in idiopathic PPFE patients, but not in secondary PPFE patients. Conclusions: The frequency and prognostic impact of UIP-pattern ILD differed between idiopathic and secondary PPFE patients. Lung transplantation should be considered in secondary PPFE patients with low diffusing capacity of the lungs for carbon monoxide (DLCO) regardless of lower-lobe ILD pattern.
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影响因子：
4.3
作者：
Jacob, Joseph;Odink, Arlette;Wells, Athol U.
通讯作者：
Wells, Athol U.
影响因子：
5.8
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
通讯作者：
Date H
影响因子：
--
作者：
van den Hoogen, Frank;Khanna, Dinesh;Fransen, Jaap;Johnson, Sindhu R.;Baron, Murray;Tyndall, Alan;Matucci-Cerinic, Marco;Naden, Raymond P.;Medsger, Thomas A., Jr.;Carreira, Patricia E.;Riemekasten, Gabriela;Clements, Philip J.;Denton, Christopher P.;Distler, Oliver;Allanore, Yannick;Furst, Daniel E.;Gabrielli, Armando;Mayes, Maureen D.;van Laar, Jacob M.;Seibold, James R.;Czirjak, Laszlo;Steen, Virginia D.;Inanc, Murat;Kowal-Bielecka, Otylia;Mueller-Ladner, Ulf;Valentini, Gabriele;Veale, Douglas J.;Vonk, Madelon C.;Walker, Ulrich A.;Chung, Lorinda;Collier, David H.;Csuka, Mary Ellen;Fessler, Barri J.;Guiducci, Serena;Herrick, Ariane;Hsu, Vivien M.;Jimenez, Sergio;Kahaleh, Bashar;Merkel, Peter A.;Sierakowski, Stanislav;Silver, Richard M.;Simms, Robert W.;Varga, John;Pope, Janet E.
通讯作者：
Pope, Janet E.
影响因子：
3.7
作者：
Enomoto Y;Nakamura Y;Colby TV;Johkoh T;Sumikawa H;Nishimoto K;Yoshimura K;Matsushima S;Oyama Y;Hozumi H;Kono M;Fujisawa T;Enomoto N;Inui N;Iwashita T;Suda T
通讯作者：
Suda T
影响因子：
6.9
作者：
Baroke, Eva;Heussel, Claus Peter;Kreuter, Michael
通讯作者：
Kreuter, Michael