Department of Radiology, The Affiliated Children's Hospital, Capital Institute of Pediatrics, Beijing, China.
Department of Rheumatology, The Affiliated Children's Hospital, Capital Institute of Pediatrics, Beijing, China.
Can Respir J. 2023 Mar 27;2023:5607473. doi: 10.1155/2023/5607473. eCollection 2023.
There is no radiological measurement to estimate the severity of pediatrics juvenile dermatomyositis (JDM) with interstitial lung disease (ILD). We validated the effectiveness of CT scoring assessment in JDM patients with ILD.
To establish a CT scoring system and calculate CT scores in JDM patients with ILD and to determine its reliability and the correlation with Krebs von den Lungen-6 (KL-6).
The study totally enrolled 46 JDM-ILD patients and 16 JDM without ILD (non-ILD, NILD) patients. The chest CT images (7.0 ± 3.6 years; 32 male and 30 female) were all analyzed. CT scores of six lung zones were retrospectively calculated, included image pattern score and distribution range score. Image pattern score was defined as follows: increased broncho-vascular bundle (1 point); ground glass opacity (GGO) (2 points); consolidation (3 points); GGO with bronchiectasis (4 points); consolidation with bronchiectasis (5 points); and honeycomb lung (6 points). Distribution range score was defined as no infiltrate (0 point); <30% (1 point); 30%-60% (2 points); and ≥60% (3 points). Two pediatric radiologists reviewed all CT images independently. The ROC curve was established, and the optimal cutoff score for severity discrimination was set.
The agreement between two observers was excellent, and the ICC was 0.930 (95% CI 0.882-0.959, < 0.01). CT score and KL-6 level had a positive linear correlation ( = 0.784, < 0.01). However, the correlation between CT scores of different lung zone and KL-6 level was different. The KL-6 cut off level suggested for JDM with ILD was 209.0 U/ml, with 73.9% sensitivity and 87.5% specificity, and the area under curve was (AUC) 0.864 ( < 0.01).
The CT scoring system we established, as a semiquantitative method, can effectively evaluate ILD in JDM-PM patients and provide reliable evidence for treatment.
目前尚无影像学测量方法可用于评估儿科皮肌炎（JDM）合并间质性肺病（ILD）的严重程度。我们验证了 CT 评分评估在 JDM 合并 ILD 患者中的有效性。
建立 JDM 合并 ILD 患者的 CT 评分系统并计算 CT 评分，评估其可靠性及与 Krebs von den Lungen-6（KL-6）的相关性。
本研究共纳入 46 例 JDM-ILD 患者和 16 例 JDM 无 ILD（非 ILD，NILD）患者（胸部 CT 图像，平均年龄 7.0±3.6 岁；男 32 例，女 30 例）。回顾性计算 6 个肺区的 CT 评分，包括图像模式评分和分布范围评分。图像模式评分定义如下：支气管血管束增粗（1 分）；磨玻璃影（GGO）（2 分）；实变（3 分）；GGO 合并支气管扩张（4 分）；实变合并支气管扩张（5 分）；蜂窝肺（6 分）。分布范围评分定义为：无浸润（0 分）；＜30%（1 分）；30%-60%（2 分）；≥60%（3 分）。两名儿科放射科医生独立回顾所有 CT 图像。绘制 ROC 曲线，确定用于严重程度区分的最佳截断评分。
两名观察者之间的一致性极好，ICC 为 0.930（95%CI 0.882-0.959，<0.01）。CT 评分与 KL-6 水平呈正线性相关（r=0.784，<0.01）。然而，不同肺区 CT 评分与 KL-6 水平之间的相关性不同。建议 JDM 合并 ILD 的 KL-6 截断值为 209.0 U/ml，其敏感性为 73.9%，特异性为 87.5%，曲线下面积（AUC）为 0.864（<0.01）。
我们建立的 CT 评分系统作为一种半定量方法，可以有效评估 JDM 合并 PM 患者的 ILD，并为治疗提供可靠证据。
