BREATHE, Department of Chronic Diseases and Metabolism, KU Leuven, B-3000, Leuven, Belgium; Department of Respiratory Diseases, University Hospitals Leuven, B-3000, Leuven, Belgium.
Division of Rheumatology, University Hospitals Leuven, B-3000, Leuven, Belgium.
Respir Med. 2021 Nov-Dec;189:106626. doi: 10.1016/j.rmed.2021.106626. Epub 2021 Oct 1.
Randomized placebo-controlled trials demonstrated the efficacy of antifibrotic treatment in non-IPF progressive fibrosing ILD (fILD). Currently, there is no consensus on how progression should be defined and clinical data of non-IPF fILD patients in a real-world setting are scarce. Non-IPF fILD patients presenting at the University Hospitals Leuven between 2012 and 2016 were included. Different definitions of progression according to the selection criteria of the INBUILD, RELIEF and the uILD study were retrospectively evaluated at every hospital visit. Univariate and multivariate analyses were performed to identify predictors of progression and mortality. The study cohort comprised 120 patients; 68.3%, 54.2% and 65.8% had progressive disease based on the INBUILD, RELIEF and uILD study, respectively. A large overlap of progressive fILD patients according to the different clinical trials was observed. Median transplant-free survival time of progressive fILD patients was 3.9, 3.9, 3.8 years and the median time-to-progression after diagnosis was 2.0, 3.1 and 2.3 years according to the INBUILD, RELIEF and uILD study, respectively. We identified several predictors of mortality, but only an underlying diagnosis of HP and uILD was independently associated with progression. Our data show a high prevalence of progressive fibrosis among non-IPF fILD patients and a discrepancy between predictors of mortality and progression. Mortality rate in fILD is high and the identification of progressive disease is only made late during the disease course. Moreover, future treatment decisions will be based upon disease behavior. Therefore, more predictors of progressive disease are needed to guide treatment decisions in the future.
随机安慰剂对照试验证明了抗纤维化治疗在非特发性肺纤维化（IPF）进行性纤维化间质性肺疾病（fILD）中的疗效。目前，对于如何定义进展尚无共识，而且在真实环境中，非 IPF fILD 患者的临床数据也很少。该研究纳入了 2012 年至 2016 年期间在鲁汶大学医院就诊的非 IPF fILD 患者。回顾性评估了根据 INBUILD、RELIEF 和 uILD 研究选择标准的不同进展定义，并在每次就诊时进行单变量和多变量分析，以确定进展和死亡的预测因素。该研究队列包括 120 例患者；根据 INBUILD、RELIEF 和 uILD 研究，分别有 68.3%、54.2%和 65.8%的患者为进展性疾病。根据不同临床试验，进展性 fILD 患者存在较大重叠。进展性 fILD 患者的中位无移植生存时间分别为 3.9、3.9、3.8 年，根据 INBUILD、RELIEF 和 uILD 研究，诊断后进展时间分别为 2.0、3.1 和 2.3 年。我们确定了几个死亡的预测因素，但只有潜在的 HP 和 uILD 诊断与进展独立相关。我们的数据显示，非 IPF fILD 患者中存在较高的纤维化进展患病率，且死亡率和进展的预测因素存在差异。fILD 的死亡率较高，而且仅在疾病过程中晚期才发现进行性疾病。此外，未来的治疗决策将基于疾病行为。因此，需要更多的进展性疾病预测因素来指导未来的治疗决策。
