Cyclophosphamide for interstitial lung disease-associated acute respiratory failure: mortality, clinical response and radiological characteristics.

Cyclophosphamide for interstitial lung disease-associated acute respiratory failure: mortality, clinical response and radiological characteristics.
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DOI:
10.1186/s12890-021-01615-2
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发表时间:
2021-07-28
影响因子:
3.1
通讯作者:
Bonta PI
Bonta PI
中科院分区:
医学3区
文献类型:
--
作者:
van Gemert JP;van den Berk IAH;Nossent EJ;Heunks LMA;Jonkers RE;Vlaar AP;Bonta PI

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间质性肺病(ILD)合并急性呼吸衰竭(ARF)患者的治疗具有挑战性,指导此类治疗的文献很少。据报道,伴有 ARF 的 ILD 患者的院内死亡率很高(62-66%)。环磷酰胺被认为是类固醇难治性 ILD 相关 ARF 的二线治疗方法。本研究的首要目的是评估接受环磷酰胺治疗的 ILD 相关 ARF 患者的院内死亡率。第二个目标是比较幸存者和非幸存者之间的计算机断层扫描 (CT) 模式以及生理和呼吸机参数。对 2016 年 2 月至 2017 年 10 月期间接受环磷酰胺治疗的 ILD 相关 ARF 患者进行回顾性分析。患者被分为三个亚组:结缔组织病 (CTD) 相关 ILD、其他 ILD 或血管炎。对整个队列和这些亚组的院内死亡率进行了评估。使用生理和呼吸机参数确定临床反应:环磷酰胺治疗前后的序贯器官衰竭评估评分 (SOFA)、PaO2/FiO2 (P/F) 比值和动态顺应性 (Cdyn)。对以下 CT 特征进行量化:毛玻璃混浊 (GGO) 比例、网状比例、实质疾病的总体范围和纤维化粗糙度评分。包括十五名患者。总体院内死亡率为40%。 CTD 相关 ILD、其他 ILD 和血管炎的院内死亡率分别为 20%、57% 和 33%。非幸存者中的 GGO 比例(71% 比 45%)更高。幸存者和非幸存者之间的 SOFA 评分、P/F 比或 Cdyn 没有显着差异。然而,在环磷酰胺治疗后 3 天,幸存者的 P/F 比值从 129 mmHg 增加到 220 mmHg,Cdyn 从 75 mL/cmH2O 增加到 92 mL/cmH2O。在非幸存者中,P/F 比率几乎没有变化 (113–114 mmHg),Cdyn 甚至下降 (27–20 mL/cmH2O)。在这项研究中,我们发现接受环磷酰胺治疗的 ILD 相关 ARF 患者的死亡率为 40%。结缔组织病相关的 ILD 和血管炎与较低的死亡风险相关。在非幸存者中,CT GGO 比例明显更高。环磷酰胺治疗 3 天后,幸存者的 P/F 比值和 Cdyn 有所增加。在线版本包含可在 10.1186/s12890-021-01615-2 获取的补充材料。
Treatment for interstitial lung disease (ILD) patients with acute respiratory failure (ARF) is challenging, and literature to guide such treatment is scarce. The reported in-hospital mortality rates of ILD patients with ARF are high (62–66%). Cyclophosphamide is considered a second-line treatment in steroid-refractory ILD-associated ARF. The first aim of this study was to evaluate the in-hospital mortality in patients with ILD-associated ARF treated with cyclophosphamide. The second aim was to compare computed tomographic (CT) patterns and physiological and ventilator parameters between survivors and non-survivors. Retrospective analysis of patients with ILD-associated ARF treated with cyclophosphamide between February 2016 and October 2017. Patients were categorized into three subgroups: connective tissue disease (CTD)-associated ILD, other ILD or vasculitis. In-hospital mortality was evaluated in the whole cohort and in these subgroups. Clinical response was determined using physiological and ventilator parameters: Sequential Organ Failure Assessment Score (SOFA), PaO2/FiO2 (P/F) ratio and dynamic compliance (Cdyn) before and after cyclophosphamide treatment. The following CT features were quantified: ground-glass opacification (GGO) proportion, reticulation proportion, overall extent of parenchymal disease and fibrosis coarseness score. Fifteen patients were included. The overall in-hospital mortality rate was 40%. In-hospital mortality rates for CTD-associated ILD, other ILD and vasculitis were 20, 57, and 33%, respectively. The GGO proportion (71% vs 45%) was higher in non-survivors. There were no significant differences in the SOFA score, P/F ratio or Cdyn between survivors and non-survivors. However, in survivors the P/F ratio increased from 129 to 220 mmHg and Cdyn from 75 to 92 mL/cmH2O 3 days after cyclophosphamide treatment. In non-survivors the P/F ratio hardly changed (113–114 mmHg) and Cdyn even decreased (27–20 mL/cmH2O). In this study, we found a mortality rate of 40% in patients treated with cyclophosphamide for ILD-associated ARF. Connective tissue disease-associated ILD and vasculitis were associated with a lower risk of death. In non-survivors, the CT GGO proportion was significantly higher. The P/F ratio and Cdyn in survivors increased after 3 days of cyclophosphamide treatment. The online version contains supplementary material available at 10.1186/s12890-021-01615-2.
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