Background

Connective tissue disease associated interstitial lung disease (CTD-ILD) is associated with decreased quality of life and high mortality risk. Outcome and treatment response is unpredictable. This study aimed to identify clinical predictors for CTD-ILD with poor outcome.

Methods

We performed a retrospective single centre cohort study in outpatients with CTD-ILD seen between 2004 and 2018. Clinical and biochemical data, pulmonary function tests (PFT) and high-resolution computed tomography (HRCT) results were analysed. Overall survival and progressive fibrosing ILD (PF-ILD, defined as a significant deterioration of PFT or HRCT) after two years of follow-up were assessed.

Results

In total, 150 patients with CTD-ILD were included. Thirty (20%) deaths occurred during a median follow-up of 40 months (IQR 27.3–60.8), which were attributed to pulmonary infection in six (4%), respiratory failure due to PF-ILD in ten (7%) and due to other causes in fourteen patients. PF-ILD occurred in 76 (50.7%) patients and was associated with poor overall survival (adjusted HR 5.73, 95%CI 1.17–28.11). Age, smoking, C-reactive protein, and steroid-use were independently associated with increased mortality risk as well. Furthermore, patients with diabetes mellitus (adjusted OR 4.52, 95%CI 1.10–18.51), steroid-use (adjusted OR 2.26, 95%CI 1.04–4.93), and a fibrotic HRCT pattern at baseline (adjusted OR 3.11, 95%CI 1.15–8.38) had a higher risk of PF-ILD.

Conclusion

PF-ILD is associated with increased mortality in patients with CTD-ILD. Patients with a fibrotic HRCT pattern at baseline, diabetes mellitus and steroid-use have a higher risk of developing PF-ILD.

中文翻译：

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结缔组织病相关间质性肺病患者进行性纤维化的预测因子

背景

结缔组织病相关间质性肺病 (CTD-ILD) 与生活质量下降和高死亡率风险相关。结果和治疗反应是不可预测的。本研究旨在确定预后不良的 CTD-ILD 的临床预测因子。

方法

我们对 2004 年至 2018 年间的 CTD-ILD 门诊患者进行了一项回顾性单中心队列研究。分析了临床和生化数据、肺功能检查 (PFT) 和高分辨率计算机断层扫描 (HRCT) 结果。评估了两年随访后的总生存率和进行性纤维化 ILD（PF-ILD，定义为 PFT 或 HRCT 显着恶化）。

结果

总共包括 150 名 CTD-ILD 患者。在 40 个月的中位随访期间（IQR 27.3-60.8）发生了 30 例（20%）死亡，其中 6 例（4%）归因于肺部感染，10 例（7%）归因于 PF-ILD 引起的呼吸衰竭和由于其他原因，十四名患者。PF-ILD 发生在 76 名 (50.7%) 患者中，并与较差的总生存期相关（调整后的 HR 5.73, 95% CI 1.17–28.11）。年龄、吸烟、C反应蛋白和类固醇使用也与死亡风险增加独立相关。此外，患有糖尿病（校正 OR 4.52, 95%CI 1.10-18.51）、使用类固醇（校正 OR 2.26, 95%CI 1.04-4.93）和基线时存在纤维化 HRCT 模式（校正 OR 3.11, 95%CI 1.15–8.38) 具有更高的 PF-ILD 风险。

结论

PF-ILD 与 CTD-ILD 患者死亡率增加有关。基线时具有纤维化 HRCT 模式、糖尿病和使用类固醇的患者发生 PF-ILD 的风险更高。