系统性硬化症中的胸膜实质纤维弹性症:患病率和预后影响摩根富林明-欧洲呼吸杂志》乔和J - 10.1183/13993003.02135 -2019欧元六世- 56 - 1 SP - 1902135 AU Bonifazi玛蒂娜盟——Sverzellati尼古拉AU - Negri,伊娃盟——雅各,约瑟AU - Egashira,恭子盟,莫泽乔安娜盟——Piciucchi萨拉非盟-梅,费德里科•AU - De Lauretis安吉洛AU - Visca,蒂娜AU -吴作栋,妮可盟——Bonini Matteo AU - Cirilli,劳拉·拉维奇亚盟-卡洛非盟-蔡,菲利克斯盟——Kouranos Vasileios AU - Margaritopoulos,乔治AU - Kokosi,玛丽亚AU -马赫,托比·m . AU - Gasparini斯特凡诺盟——加,阿曼德盟-威尔斯,Athol美国的非盟- Renzoni,伊丽莎白·a·Y1 - 2020/07/01 UR - //www.qdcxjkg.com/content/56/1/1902135.abstract N2 -间质性肺病(ILD)在系统性硬化症(SSc)是一种发病率和死亡率的主要原因,主要表现为非特异性间质性肺炎。对于胸膜实质纤维弹性症(PPFE)的患病率所知甚少，PPFE是一种影响内脏胸膜和胸膜下实质的特殊实体。我们开始评估两大群SSc患者PPFE的患病率，并评估其对生存和功能衰退的影响。共有359名来自两个不同国家(英国和意大利)的转诊中心的SSc患者被包括在内。第一个可用的高分辨率计算机断层扫描是由两名不了解临床信息的放射科医生独立评估的，以量化ILD的范围，独立的支气管异常，和肺叶PPFE的百分比在4点分类量表上。不一致的分数由第三个评分员判定。PPFE范围进一步分为有限(≤2/18)和广泛(>2/18)。将结果与功能衰退和死亡率进行比较。合并SSc人群PPFE的总体患病率为18%(11%为广泛PPFE)，两组间无显著差异。 PPFE was significantly linked to free-standing bronchial abnormalities (61% versus 25% in PPFE versus 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.We provide a thorough assessment of pleuroparenchymal fibroelastosis (PPFE) prevalence, severity and clinical impact in two large cohorts of scleroderma patients (total n=359). PPFE was present in 18% of patients and independently predicted mortality. https://bit.ly/2xDVvG3 ER -