抗合成酶综合征相关间质性肺病患者进行性肺纤维化的预测。
Prediction of progressive pulmonary fibrosis in patients with anti-synthetase syndrome-associated interstitial lung disease.
机构信息
Department of Key Laboratory of Ningxia Stem Cell and Regenerative Medicine, Institute of Medical Sciences, General Hospital of Ningxia Medical University, Yinchuan, 750004, Ningxia, China.
Department of Pulmonary and Critical Care Medicine, General Hospital of Ningxia Medical University, Yinchuan, 750004, Ningxia, China.
出版信息
Clin Rheumatol. 2023 Jul;42(7):1917-1929. doi: 10.1007/s10067-023-06570-3. Epub 2023 Mar 17.
OBJECTIVE
Interstitial lung disease (ILD) is a common extramuscular manifestation of the anti-synthetase syndrome (ASS). Patients with ASS-ILD are at risk in developing a progressive fibrosing phenotype despite appropriate treatments. This study investigated the risk factors and the predictive value of multiple risk factors for progressive pulmonary fibrosis (PPF) in patients with ASS-ILD.
METHODS
Ninety patients with a diagnosis of ASS and evidence of ILD on high-resolution computed tomography (HRCT) were recruited. Among them, 72 participants completed follow-up for more than 12 months. These patients were further divided into a PPF-ASS group (n = 18) and a non-PPF-ASS group (n = 54). Logistic regression analysis was performed to investigate the risk factors for PPF. The predictive value of the combined risk factors for predicting PPF were analyzed by a ROC curve.
RESULTS
The PPF-ASS group had a higher rate of positive non-Jo-1 antibodies, a significantly higher neutrophil-to-lymphocyte ratio (NLR) and serum lactate dehydrogenase (LDH), and a significantly lower PaO/FiO ratio and diffusing capacity for carbon monoxide (DLCO%pred) than the non-PPF-ASS group. In addition, elevated serum Krebs von den Lungen-6 (KL-6) level and reticular opacities were significantly more common, and corticosteroid monotherapy at onset was administered more frequently in the PPF-ASS group. The median duration of follow-up was 37.4 months, survival was poorer in the PPF-ASS group, and the overall survival was 88.9%. Multivariate regression analysis further revealed that positive non-Jo-1 antibodies, NLR, and KL-6 were independent risk factors for PPF. These combined indexes had good accuracy (area under the curve = 0.874) in predicting PPF in patients with ASS-ILD.
CONCLUSION
Positive non-Jo-1 antibodies, NLR, and serum KL-6 are independent risk factors for PPF in patients with ASS-ILD. Monitoring these markers can potentially predict PPF in this group of patients. Key Points • Positive non-Jo-1 antibodies, NLR, and serum KL-6 are independent risk factors associated with PPF in patients with ASS-ILD. • Monitoring non-Jo-1 antibodies, NLR, and serum KL-6 can potentially predict PPF in patients with ASS-ILD.
目的
间质性肺病（ILD）是抗合成酶综合征（ASS）的常见肌外表现。尽管接受了适当的治疗，ASS-ILD 患者仍有发展为进行性纤维化表型的风险。本研究旨在探讨 ASS-ILD 患者发生进行性肺纤维化（PPF）的多种危险因素及其预测价值。
方法
共纳入 90 例 ASS 诊断明确且高分辨率 CT（HRCT）显示 ILD 的患者。其中 72 例患者完成了超过 12 个月的随访。这些患者进一步分为 PPF-ASS 组（n=18）和非 PPF-ASS 组（n=54）。采用 Logistic 回归分析探讨 PPF 的危险因素。通过 ROC 曲线分析联合危险因素对 PPF 的预测价值。
结果
PPF-ASS 组的非-Jo-1 抗体阳性率、中性粒细胞与淋巴细胞比值（NLR）和血清乳酸脱氢酶（LDH）显著更高，动脉血氧分压与吸入氧分数比值（PaO/FiO）和一氧化碳弥散量（DLCO%pred）显著更低。此外，PPF-ASS 组血清 Krebs von den Lungen-6（KL-6）水平升高和网状阴影更为常见，且发病时更常接受皮质类固醇单药治疗。中位随访时间为 37.4 个月，PPF-ASS 组患者的生存率更差，总生存率为 88.9%。多变量回归分析进一步表明，非-Jo-1 抗体阳性、NLR 和 KL-6 是非 PPF-ASS 患者发生 PPF 的独立危险因素。这些联合指标对 ASS-ILD 患者 PPF 的预测具有较好的准确性（曲线下面积=0.874）。
结论
非-Jo-1 抗体阳性、NLR 和血清 KL-6 是非 ASS-ILD 患者发生 PPF 的独立危险因素。监测这些标志物可能有助于预测该组患者的 PPF。关键点•非-Jo-1 抗体阳性、NLR 和血清 KL-6 是 ASS-ILD 患者发生 PPF 的独立危险因素。•监测非-Jo-1 抗体、NLR 和血清 KL-6 可能有助于预测 ASS-ILD 患者的 PPF。
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