Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
Health Administration Center, Hamamatsu University School of Medicine, 1-20-1 Handayama, Hamamatsu, 431-3192, Japan.
Respir Res. 2024 Sep 28;25(1):346. doi: 10.1186/s12931-024-02982-0.
Interstitial pneumonia with autoimmune features (IPAF), which does not meet any of the criteria for connective tissue diseases (CTD), has been attracting an attention in patients with idiopathic interstitial pneumonia (IIP). However, the biomarkers that reflect the clinical course of these patients have not been fully elucidated.
To identify useful serum biomarkers reflecting CTD-related features and favorable prognoses in patients with IIP.
This was a post hoc analysis of a prospective and multicenter cohort study between 2015 and 2020. Newly diagnosed patients with IIP were consecutively enrolled, and 74 autoimmune features and autoantibodies were comprehensively checked during IIP diagnosis. Serum levels of CXCL10, CXCL1, CCL2, BAFF, angiopoietin-2, and leptin were evaluated at the time of IIP diagnosis.
Two hundred twenty-two patients (159 men and 63 women) with IIP were enrolled. The median observation duration was 36 months. The median age was 71 years old, and median %forced vital capacity (FVC) was 84.1% at the time of IIP diagnosis. The proportion of patients who met the classification criteria for IPAF was 11.7%. In patients with high serum CXCL10, changes in both %FVC and %diffusion lung capacity for carbon monoxide at one year were significantly higher than those in patients with low CXCL10 (p = 0.014 and p = 0.009, respectively), whereas these changes were not significant for other chemokines and cytokines. High CXCL10 levels were associated with acute/subacute onset (p < 0.001) and the diagnosis of nonspecific interstitial pneumonia with organizing pneumonia overlap (p = 0.003). High CXCL10 levels were related to a higher classification of IPAF (relative risk for IPAF was 3.320, 95%CI: 1.571-7.019, p = 0.003) and lower classification of progressive pulmonary fibrosis (PPF; relative risk for PPF was 0.309, 95%CI: 0.100-0.953, p = 0.027) compared to those with low CXCL10. Finally, survival was higher in patients with IPF and high CXCL10 (p = 0.044), and high CXCL10 was a significant prognostic factor in multivariate Cox proportional hazards models (hazard ratio 0.368, p = 0.005).
High serum levels of CXCL10 are associated with CTD-related features, the favorable clinical course, and survival in patients with IIP, especially IPF.
Not applicable.
具有自身免疫特征的间质性肺炎（IPAF）不符合任何结缔组织疾病（CTD）的标准，在特发性间质性肺炎（IIP）患者中引起了关注。然而，反映这些患者临床病程的生物标志物尚未得到充分阐明。
确定反映 IIP 患者 CTD 相关特征和良好预后的有用血清生物标志物。
这是一项 2015 年至 2020 年期间进行的前瞻性多中心队列研究的事后分析。连续纳入新诊断的 IIP 患者，并在 IIP 诊断期间全面检查 74 种自身免疫特征和自身抗体。在 IIP 诊断时评估血清 CXCL10、CXCL1、CCL2、BAFF、血管生成素-2 和瘦素的水平。
共纳入 222 例（159 名男性和 63 名女性）IIP 患者。中位观察时间为 36 个月。中位年龄为 71 岁，IPF 诊断时中位用力肺活量（FVC）%为 84.1%。符合 IPAF 分类标准的患者比例为 11.7%。在血清 CXCL10 水平较高的患者中，一年时 FVC 和一氧化碳弥散量的变化明显高于 CXCL10 水平较低的患者（p=0.014 和 p=0.009，分别），而其他趋化因子和细胞因子的变化则不明显。高 CXCL10 水平与急性/亚急性发作（p<0.001）和非特异性间质性肺炎伴机化性肺炎重叠诊断（p=0.003）相关。高 CXCL10 水平与更高的 IPAF 分类（IPAF 的相对风险为 3.320，95%CI：1.571-7.019，p=0.003）和较低的进展性肺纤维化（PPF）分类相关（PPF 的相对风险为 0.309，95%CI：0.100-0.953，p=0.027）与 CXCL10 水平较低的患者相比。最后，在 IPF 和高 CXCL10 患者中，生存率更高（p=0.044），多变量 Cox 比例风险模型显示高 CXCL10 是一个显著的预后因素（风险比 0.368，p=0.005）。
血清 CXCL10 水平升高与 IIP 患者的 CTD 相关特征、良好的临床病程和生存相关，尤其是与 IPF 相关。
不适用。
