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

BMC Pulmonary Medicine

Alexander P. Vlaar¹  Johanna P. van Gemert¹  Leo M. A. Heunks²  Inge A. H. van den Berk³  Rene E. Jonkers⁴  Peter I. Bonta⁴  Esther J. Nossent⁵ 

[1] Department of Intensive Care Medicine, Amsterdam University Medical Center, Location AMC, University of Amsterdam;Department of Intensive Care Medicine, Amsterdam University Medical Center, Location VUMC, Vrije Universiteit;Department of Radiology, Amsterdam University Medical Center, Location AMC, University of Amsterdam;Department of Respiratory Medicine, Amsterdam University Medical Center, Location AMC, University of Amsterdam;Department of Respiratory Medicine, Amsterdam University Medical Center, Location VUMC, Vrije Universiteit;

关键词: Interstitial lung disease;    Acute respiratory failure;    Cyclophosphamide;    Ground-glass opacification;   

DOI  :  10.1186/s12890-021-01615-2

来源: DOAJ

【 摘 要 】

Abstract Background 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. Methods 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. Results 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). Conclusion 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.

【 授权许可】

Unknown