Interstitial Lung Diseases Center of Excellence, Department of Pulmonology, St Antonius Hospital, Koekoekslaan 1, 3435 CM, Nieuwegein, The Netherlands.
Interstitial Lung Diseases Center of Excellence, Department of Radiology, St Antonius Hospital, Nieuwegein, The Netherlands.
Respir Res. 2022 Jun 25;23(1):169. doi: 10.1186/s12931-022-02094-7.
Advanced pulmonary sarcoidosis causes significant morbidity and can lead to death. Large trials demonstrated efficacy of antifibrotics in patients with progressive fibrosing interstitial lung diseases (PF-ILD), including a few with sarcoidosis. To date, little is known about this progressive fibrosing phenotype in sarcoidosis. Diffusion capacity of carbon monoxide (DLCO) may be a useful functional marker to screen for advanced pulmonary sarcoidosis. In this study, we describe a cohort with advanced pulmonary sarcoidosis and we gain insights in the progressive fibrosing phenotype in sarcoidosis.
Patients with sarcoidosis and a DLCO < 50% predicted were included in this retrospective cohort study. First measurement of DLCO < 50% predicted was the baseline. Lung function data, HRCT, pulmonary hypertension (PH) and mortality were collected. Patients with > 10% fibrosis on HRCT meeting the criteria for ILD-progression within 24 months were labelled as PF-ILD. With Cox-regression analysis predictors of mortality were established.
106 patients with a DLCO < 50% predicted were included. Evolution of forced vital capacity (FVC) varied widely between patients from - 34% to + 45% after 2 years follow-up, whereas change in DLCO varied between - 11% and + 26%. Fourteen patients (15%) met the PF-ILD criteria, of whom 6 (43%) died within 10 years versus 10 (13%) in the non PF-ILD group (p = 0.006). PH was present 12 (11%), 56 (53%) demonstrated > 10% fibrosis on HRCT. Independent predictors of mortality and lung transplantation in the whole cohort are PH, PF-ILD and UIP-like pattern.
In conclusion, within this group with advanced pulmonary sarcoidosis disease course varied widely from great functional improvement to death. PF-ILD patients had higher mortality rate than the mortality in the overall pulmonary sarcoidosis group. Future research should focus on the addition of antifibrotics in these patients. Trial registration retrospectively registered.
晚期肺结节病会导致严重的发病率，并可能导致死亡。大型试验表明，抗纤维化药物在进展性肺纤维化间质性肺疾病（PF-ILD）患者中是有效的，其中包括一些结节病患者。迄今为止，人们对结节病中的这种进行性纤维化表型知之甚少。一氧化碳弥散量（DLCO）可能是筛查晚期肺结节病的有用功能标志物。在这项研究中，我们描述了一组患有晚期肺结节病的患者，并深入了解了结节病中的进行性纤维化表型。
纳入了 DLCO < 50%预测值的结节病患者进行这项回顾性队列研究。首次 DLCO < 50%预测值的测量值作为基线。收集肺功能数据、高分辨率计算机断层扫描（HRCT）、肺动脉高压（PH）和死亡率。在 24 个月内符合间质性肺病进展标准的 HRCT 上有 > 10%纤维化的患者被标记为 PF-ILD。通过 Cox 回归分析确定死亡率的预测因素。
共纳入 106 例 DLCO < 50%预测值的患者。在 2 年的随访中，患者的用力肺活量（FVC）变化范围从-34%到+45%，而 DLCO 的变化范围从-11%到+26%。14 名患者（15%）符合 PF-ILD 标准，其中 6 名（43%）在 10 年内死亡，而非 PF-ILD 组为 10 名（13%）（p=0.006）。存在 PH 的患者有 12 名（11%），56 名（53%）在 HRCT 上有 > 10%的纤维化。整个队列中，死亡率和肺移植的独立预测因素是 PH、PF-ILD 和 UIP 样模式。
在这组患有晚期肺结节病的患者中，疾病的发展过程从功能显著改善到死亡不等。PF-ILD 患者的死亡率高于总体肺结节病组的死亡率。未来的研究应集中在这些患者中添加抗纤维化药物。试验注册为回顾性注册。
