Abstract
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%versus25.% in PPFEversus没有ppfe;P <0.0001)并更差的存活率,独立于严重程度或短期肺功能变化(HR 1.89,95%CI 1.10-3.25; P = 0.005)。
在目前的研究中,我们在迄今为止发布的最大SSC科目中的PPFE患病率和临床影响提供了详尽的描述。由于其显着的预后意义,应仔细考虑PPFE存在。
Footnotes
This manuscript has recently been accepted for publication in theEuropean Respiratory Journal。在我们的制作团队中的复制和排版之前,它在其接受的表单中发布。在这些生产过程完成后,作者已批准所产生的证据,这篇文章将转向最新问题ERJonline. Please open or download the PDF to view this article.
兴趣冲突:博尼比亚博士向罗什·尼尔海姆报告个人费用,罗氏的个人费用,在提交的工作之外。
Conflict of interest: Dr. SVERZELLATI reports personal fees from BOEHRINGER-INGELHEIM, personal fees from ROCHE, outside the submitted work.
Conflict of interest: Dr. Negri has nothing to disclose.
Conflict of interest: Dr. Jacob reports personal fees from Roche, personal fees from Boehringer Ingelheim, grants from GlaxoSmithKline, outside the submitted work.
Conflict of interest: Dr. EGASHIRA reports personal fees and non-financial support from SHIONOGI, personal fees and non-financial support from Boehringer Ingelheim Japan, personal fees and non-financial support from Bayer Yakuhin, personal fees and non-financial support from EISAI, personal fees and non-financial support from DAIICHI SANKYO, personal fees and non-financial support from AstraZeneca, non-financial support from KYORIN, outside the submitted work.
Conflict of interest: Dr. Moser has nothing to disclose.
Conflict of interest: Dr. Piciucchi has nothing to disclose.
利益冲突:梅博士没有披露。
利益冲突:De Lauretis博士无需披露。
Conflict of interest: Dr. VISCA has nothing to disclose.
Conflict of interest: Dr. Goh has nothing to disclose.
Conflict of interest: Dr. Bonini has nothing to disclose.
Conflict of interest: Dr. CIRILLI has nothing to disclose.
利益冲突:拉维基亚博士无需披露。
Conflict of interest: Dr Chua has no conflict of interest to declare
Conflict of interest: Dr. Kouranos has nothing to disclose.
Conflict of interest: Dr. Margaritopoulos has nothing to disclose.
利益冲突:Kokosi博士无需披露。
Conflict of interest: TMM has, via his institution, received industry-academic funding from GlaxoSmithKline R&D and UCB and has received consultancy or speakers fees from Apellis, Astra Zeneca, Bayer, Blade Therapeutics, Boehringer Ingelheim, Bristol-Myers Squibb, Galapagos, GlaxoSmithKline R&D, Indalo, Novartis, Pliant, ProMetic, Respivnat, Roche, Samumed and UCB.
Conflict of interest: Dr. GASPARINI has nothing to disclose.
Conflict of interest: Armando Gabrielli
利益冲突:教授。Wells报告了Boehringer-Ingelheim的个人费用,罗氏的个人费用,拜耳的个人费用,在提交的工作之外。
利益冲突:RENZONI博士个人报告fees from BOEHRINGER-INGELHEIM, personal fees from ROCHE, outside the submitted work.
- ReceivedNovember 3, 2019.
- 公认2020年3月25日。
- 版权所有©ers 2020