Bai Jiu-Wu, Huang Jian-Nan, Shi Shen-Yun, Ge Ai, Lu Hai-Wen, Sun Xiao-Li, Gu Shu-Yi, Liang Shuo, Cheng Ke-Bin, Tian Xin-Lun, Xiao Yong-Long, Xu Kai-Feng, Xu Jin-Fu
Department of Respiratory and Critical Care Medicine, Shanghai Pulmonary Hospital, School of Medicine, Tongji University, Shanghai, China.
Department of Respiratory and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
Front Med (Lausanne). 2023 Feb 7;10:1058001. doi: 10.3389/fmed.2023.1058001. eCollection 2023.
The high-resolution computed tomography (HRCT) score is an important component of the severity and prognosis score of pulmonary alveolar proteinosis (SPSP). However, the HRCT score in SPSP only considers the extent of opacity, which is insufficient.
We retrospectively evaluated HRCT scores for 231 patients with autoimmune pulmonary alveolar proteinosis (APAP) from three centers of the China Alliance for Rare Diseases. The SPSPII was created based on the overall density and extent, incorporating the SPSP. The severity of APAP patients was assessed using disease severity scores (DSS), SPSP, and SPSPII to determine the strengths and weaknesses of the different assessment methods. We then prospectively applied the SPSPII to patients before treatment, and the curative effect was assessed after 3 months.
The HRCT overall density and extent scores in our retrospective analysis were higher than the extent scores in all patients and every original extent score severity group, as well as higher related to arterial partial oxygen pressure (PaO) than extent scores. The mild patients accounted for 61.9% based on DSS 1-2, 20.3% based on SPSP 1-3, and 20.8% based on SPSPII 1-3. Based on SPSP or SPSPII, the number of severe patients deteriorating was higher in the mild and moderate groups. When applied prospectively, arterial PaO differed between any two SPSPII severity groups. The alveolar-arterial gradient in PaO (P[A-a]O), % predicted carbon monoxide diffusing capacity of the lung (DLCO), and HRCT score were higher in the severe group than in the mild and moderate groups. After diagnosis, mild patients received symptomatic treatment, moderate patients received pure whole lung lavage (WLL) or granulocyte-macrophage colony-stimulating factor (GM-CSF) therapy, and severe patients received WLL and GM-CSF therapy. Importantly, the SPSPII in mild and severe groups were lower than baseline after 3 months.
The HRCT density and extent scores of patients with APAP were better than the extent score. The SPSPII score system based on smoking status, symptoms, PaO, predicted DLCO, and overall HRCT score was better than DSS and SPSP for assessing the severity and efficacy and predicting the prognosis.
ClinicalTrial.gov, identifier: NCT04516577.
高分辨率计算机断层扫描（HRCT）评分是肺泡蛋白沉积症严重程度和预后评分（SPSP）的重要组成部分。然而，SPSP中的HRCT评分仅考虑了实变范围，这是不够的。
我们回顾性评估了来自中国罕见病联盟三个中心的231例自身免疫性肺泡蛋白沉积症（APAP）患者的HRCT评分。基于总体密度和范围创建了SPSPII，纳入了SPSP。使用疾病严重程度评分（DSS）、SPSP和SPSPII评估APAP患者的严重程度，以确定不同评估方法的优缺点。然后我们前瞻性地将SPSPII应用于治疗前的患者，并在3个月后评估疗效。
我们回顾性分析中的HRCT总体密度和范围评分高于所有患者及每个原始范围评分严重程度组中的范围评分，并且与动脉血氧分压（PaO）相关的程度也高于范围评分。基于DSS 1 - 2，轻度患者占61.9%；基于SPSP 1 - 3，轻度患者占20.3%；基于SPSPII 1 - 3，轻度患者占20.8%。基于SPSP或SPSPII，轻度和中度组中病情恶化的重度患者数量更多。前瞻性应用时，任意两个SPSPII严重程度组之间的动脉PaO存在差异。重度组的肺泡 - 动脉氧分压差（P[A - a]O）、肺一氧化碳弥散量预测值（DLCO）百分比和HRCT评分均高于轻度和中度组。诊断后，轻度患者接受对症治疗，中度患者接受单纯全肺灌洗（WLL）或粒细胞 - 巨噬细胞集落刺激因子（GM - CSF）治疗，重度患者接受WLL和GM - CSF治疗。重要的是，3个月后轻度和重度组的SPSPII均低于基线水平。
APAP患者的HRCT密度和范围评分优于范围评分。基于吸烟状况、症状、PaO、预测的DLCO和总体HRCT评分的SPSPII评分系统在评估严重程度和疗效以及预测预后方面优于DSS和SPSP。
ClinicalTrial.gov，标识符：NCT04516577。
