Pleuroparenchymal fibroelastosis in systemic sclerosis: prevalence and prognostic impact.
Pleuroparenchymal fibroelastosis in systemic sclerosis: prevalence and prognostic impact.
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DOI：
10.1183/13993003.02135-2019
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发表时间：
2020-07
期刊：
影响因子：
--
通讯作者：
Renzoni EA
中科院分区：
文献类型：
--
作者：
Bonifazi M;Sverzellati N;Negri E;Jacob J;Egashira R;Moser J;Piciucchi S;Mei F;De Lauretis A;Visca D;Goh N;Bonini M;Cirilli L;La Vecchia C;Chua F;Kouranos V;Margaritopoulos G;Kokosi M;Maher TM;Gasparini S;Gabrielli A;Wells AU;Renzoni EA
系统性硬化症（SSc）中的间质性肺疾病（ILD）是发病和死亡的主要原因，主要表现为非特异性间质性肺炎。关于胸膜实质弹力纤维增生症 (PPFE) 的患病率知之甚少，PPFE 是一种影响脏层胸膜和胸膜下实质的特定实体。我们着手估计两大 SSc 患者队列中 PPFE 的患病率，并评估其对生存和功能衰退的影响。共有 359 名 SSc 患者被纳入研究，这些患者来自两个不同国家（英国和意大利）的两个转诊中心。第一个可用的高分辨率计算机断层扫描由两名不了解临床信息的放射科医生独立评估，以 4 点分类量表量化 ILD 范围、独立支气管异常和 PPFE 受累肺叶百分比。不一致的分数由第三位记分员判定。 PPFE 范围进一步分为有限 (≤2/18) 或广泛 (>2/18)。根据功能衰退和死亡率对结果进行评估。合并 SSc 人群中 PPFE 的总体患病率为 18%（广泛 PPFE 为 11%），两个队列之间没有显着差异。 PPFE 与独立支气管异常显着相关（PPFE 为 61% vs 无 PPFE 为 25%；p<0.0001），并且与较差的生存率显着相关，与 ILD 严重程度或短期肺功能变化无关（HR 1.89，95% CI 1.10-3.25；p=0.005）。在当前的研究中，我们详尽描述了迄今为止发表的最大的 SSc 受试者队列中 PPFE 的患病率和临床影响。由于 PPFE 的存在具有重要的预后意义，因此应仔细考虑其存在。
Interstitial lung disease (ILD) in systemic sclerosis (SSc) is a major cause of morbidity and mortality, mostly presenting as nonspecific interstitial pneumonia. Little is known about the prevalence of pleuroparenchymal fibroelastosis (PPFE), a specific entity affecting the visceral pleura and subpleural parenchyma. We set out to estimate PPFE prevalence in two large cohorts of SSc patients and to assess its impact on survival and functional decline. A total of 359 SSc patients, derived from two referral centers in two different countries (UK and Italy), were included. The first available high-resolution computed tomography scan was independently evaluated by two radiologists blind to clinical information, to quantify ILD extent, freestanding bronchial abnormalities, and lobar percentage involvement of PPFE on a 4-point categorical scale. Discordant scores were adjudicated by a third scorer. PPFE extent was further classified as limited (≤2/18) or extensive (>2/18). Results were evaluated against functional decline and mortality. The overall prevalence of PPFE in the combined SSc population was 18% (11% with extensive PPFE), with no substantial difference between the two cohorts. PPFE was significantly linked to free-standing bronchial abnormalities (61% vs 25% in PPFE vs no PPFE; p<0.0001) and to worse survival, independently of ILD severity or short-term lung function changes (HR 1.89, 95% CI 1.10-3.25; p=0.005). In the current study, we provide an exhaustive description of PPFE prevalence and clinical impact in the largest cohort of SSc subjects published so far. PPFE presence should be carefully considered, due to its significant prognostic implications.
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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：
--
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：
19.7
作者：
de Jong, PA;Ottink, MD;Tiddens, HAWM
通讯作者：
Tiddens, HAWM
影响因子：
24.3
作者：
Reddy, Taryn L.;Tominaga, Masaki;Nicholson, Andrew G.
通讯作者：
Nicholson, Andrew G.