RMD open · 2026
Serum KL-6 and lung ultrasound B-lines: a combined non-invasive model for screening and predicting interstitial lung disease in idiopathic inflammatory myopathy.
СтатьяНаблюдательное исследованиеНазвание и аннотация: Перевод в очереди
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Zhang Weijin, Zheng Qiongbing, Zheng Shaoyu, Du Guangzhou, Zhang Qichuan, Wu Shuhan, Huang Guoxin, Zhou Zexuan, Zheng Kedi, Lin Jianqun, Hu Shijian, Lin Qisheng, Zhuang Jinghua, Chen Qingzi, Ruaro Barbara, Hoffmann-Vold Anna-Maria, Matucci-Cerinic Marco, Furst Daniel E, Chen Shaoqi, Wang Yukai
Аннотация
BACKGROUND: Interstitial lung disease (ILD) is a major pulmonary complication of idiopathic inflammatory myopathy (IIM), where early diagnosis improves outcomes. While high-resolution CT (HRCT) remains the gold standard, its radiation exposure poses concerns. Serum Krebs von den Lungen-6 (KL-6) and lung ultrasound (LUS) B-lines offer non-invasive alternatives, though their optimal diagnostic cut-offs and combined utility for IIM-ILD remain undefined. This study aimed to establish these cut-offs, evaluate diagnostic performance and develop a clinical prediction model.
METHODS: In this single-centre observational study, 162 patients diagnosed with IIM between 2020 and 2024 were enrolled. All underwent serum KL-6 testing, and 120 received systematic 50-point LUS examinations. Using HRCT as the reference standard for ILD diagnosis, receiver operating characteristics (ROC) curve analysis was used to determine optimal cut-offs and construct a combined nomogram.
RESULTS: KL-6 levels were significantly elevated in the ILD group (n=113) compared with non-ILD (n=49). The optimal KL-6 cut-off was 553 U/mL (area under the curve (AUC)=0.895, sensitivity 69%, specificity 95.9%). For LUS B-lines number, 25 was recommended as the clinical threshold (sensitivity 98.8%). Their combination enhanced diagnostic performance (AUC=0.984). An online prediction model demonstrated strong clinical applicability. In an exploratory analysis of the rapid-progressive ILD subgroups, KL-6 and B-lines showed only modest predictive value (AUC ≈ 0.65).
CONCLUSION: KL-6 ≥553 U/mL and B-lines ≥25 are effective screening thresholds, with combined use significantly improving diagnostic accuracy. The prediction model provides a practical tool for early identification in similar clinical settings. To optimise and generalise its use, external validation in multicentre cohorts is warranted.
METHODS: In this single-centre observational study, 162 patients diagnosed with IIM between 2020 and 2024 were enrolled. All underwent serum KL-6 testing, and 120 received systematic 50-point LUS examinations. Using HRCT as the reference standard for ILD diagnosis, receiver operating characteristics (ROC) curve analysis was used to determine optimal cut-offs and construct a combined nomogram.
RESULTS: KL-6 levels were significantly elevated in the ILD group (n=113) compared with non-ILD (n=49). The optimal KL-6 cut-off was 553 U/mL (area under the curve (AUC)=0.895, sensitivity 69%, specificity 95.9%). For LUS B-lines number, 25 was recommended as the clinical threshold (sensitivity 98.8%). Their combination enhanced diagnostic performance (AUC=0.984). An online prediction model demonstrated strong clinical applicability. In an exploratory analysis of the rapid-progressive ILD subgroups, KL-6 and B-lines showed only modest predictive value (AUC ≈ 0.65).
CONCLUSION: KL-6 ≥553 U/mL and B-lines ≥25 are effective screening thresholds, with combined use significantly improving diagnostic accuracy. The prediction model provides a practical tool for early identification in similar clinical settings. To optimise and generalise its use, external validation in multicentre cohorts is warranted.