Clinical course of pleuroparenchymal fibroelastosis (PPFE) shows considerable variation among patients, but there is no established prognostic prediction model for PPFE. The prediction model was developed using retrospective data from two cohorts: our single-center cohort and a nationwide multicenter cohort involving 21 institutions. Cox regression analyses were used to identify prognostic factors. The total score was defined as the weighted sum of values for the selected variables. The performance of the prediction models was evaluated by Harrell’s concordance index (C-index). We also examined the usefulness of the gender-age-physiology (GAP) model for predicting the prognosis of PPFE patients. We examined 104 patients with PPFE (52 cases from each cohort). In a multivariate Cox analysis, a lower forced vital capacity (FVC [defined as FVC < 65%]; hazard ratio [HR], 2.23), a history of pneumothorax (HR, 3.27), the presence of a lower lobe interstitial lung disease (ILD) (HR, 2.31), and higher serum Krebs von den Lungen-6 (KL-6) levels (> 550 U/mL, HR, 2.56) were significantly associated with a poor prognosis. The total score was calculated as 1 × (FVC, < 65%) + 1 × (history of pneumothorax) + 1 × (presence of lower lobe ILD) + 1 × (KL-6, > 550 U/mL). PPFE patients were divided into three groups based on the prognostic score: stage I (0–1 points), stage II (2 points), and stage III (3–4 points). The survival rates were significantly different in each stage. The GAP stage was significantly associated with the prognosis of PPFE, but no difference was found between moderate (stage II) and severe (stage III) disease. Our new model for PPFE patients (PPFE Prognosis Score) showed better performance in the prediction of mortality in comparison to the GAP model (C-index of 0.713 vs. 0.649). Our new model for PPFE patients could be useful for predicting their prognosis.

中文翻译：

---

提出的胸膜实质弹性纤维增生预后预测评分

胸膜实质弹性纤维增生症 (PPFE) 的临床病程在患者之间表现出相当大的差异，但没有建立 PPFE 的预后预测模型。预测模型是使用来自两个队列的回顾性数据开发的：我们的单中心队列和涉及 21 个机构的全国多中心队列。Cox回归分析用于确定预后因素。总分被定义为所选变量值的加权总和。预测模型的性能通过 Harrell 的一致性指数 (C-index) 进行评估。我们还检查了性别年龄生理学 (GAP) 模型在预测 PPFE 患者预后方面的有用性。我们检查了 104 名 PPFE 患者（每个队列 52 例）。在多变量 Cox 分析中，较低的用力肺活量（FVC [定义为 FVC < 65%]；风险比 [HR], 2.23)、气胸病史 (HR, 3.27)、下叶间质性肺病 (ILD) (HR, 2.31) 和血清 Krebs von den Lungen-6 (KL-6) ) 水平 (> 550 U/mL, HR, 2.56) 与预后不良显着相关。总分计算为 1 × (FVC, < 65%) + 1 × (气胸史) + 1 × (存在下叶 ILD) + 1 × (KL-6, > 550 U/mL)。PPFE患者根据预后评分分为三组：I期（0-1分）、II期（2分）和III期（3-4分）。每个阶段的存活率都有显着差异。GAP 分期与 PPFE 的预后显着相关，但在中度（II 期）和重度（III 期）疾病之间没有发现差异。与 GAP 模型相比，我们针对 PPFE 患者的新模型（PPFE 预后评分）在死亡率预测方面表现出更好的性能（C 指数为 0.713 对 0.649）。我们针对 PPFE 患者的新模型可用于预测他们的预后。