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

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 seco...

Full description

Bibliographic Details
Main Authors: Johanna P. van Gemert, Inge A. H. van den Berk, Esther J. Nossent, Leo M. A. Heunks, Rene E. Jonkers, Alexander P. Vlaar, Peter I. Bonta
Format: Article
Language:English
Published: BMC 2021-07-01
Series:BMC Pulmonary Medicine
Subjects:
Online Access:https://doi.org/10.1186/s12890-021-01615-2
id doaj-dbc973f0316e425991c6bc0093bd4f9f
record_format Article
spelling doaj-dbc973f0316e425991c6bc0093bd4f9f2021-08-01T11:41:09ZengBMCBMC Pulmonary Medicine1471-24662021-07-0121111010.1186/s12890-021-01615-2Cyclophosphamide for interstitial lung disease-associated acute respiratory failure: mortality, clinical response and radiological characteristicsJohanna P. van Gemert0Inge A. H. van den Berk1Esther J. Nossent2Leo M. A. Heunks3Rene E. Jonkers4Alexander P. Vlaar5Peter I. Bonta6Department of Intensive Care Medicine, Amsterdam University Medical Center, Location AMC, University of AmsterdamDepartment of Radiology, Amsterdam University Medical Center, Location AMC, University of AmsterdamDepartment of Respiratory Medicine, Amsterdam University Medical Center, Location VUMC, Vrije UniversiteitDepartment of Intensive Care Medicine, Amsterdam University Medical Center, Location VUMC, Vrije UniversiteitDepartment of Respiratory Medicine, Amsterdam University Medical Center, Location AMC, University of AmsterdamDepartment of Intensive Care Medicine, Amsterdam University Medical Center, Location AMC, University of AmsterdamDepartment of Respiratory Medicine, Amsterdam University Medical Center, Location AMC, University of AmsterdamAbstract 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.https://doi.org/10.1186/s12890-021-01615-2Interstitial lung diseaseAcute respiratory failureCyclophosphamideGround-glass opacification
collection DOAJ
language English
format Article
sources DOAJ
author Johanna P. van Gemert
Inge A. H. van den Berk
Esther J. Nossent
Leo M. A. Heunks
Rene E. Jonkers
Alexander P. Vlaar
Peter I. Bonta
spellingShingle Johanna P. van Gemert
Inge A. H. van den Berk
Esther J. Nossent
Leo M. A. Heunks
Rene E. Jonkers
Alexander P. Vlaar
Peter I. Bonta
Cyclophosphamide for interstitial lung disease-associated acute respiratory failure: mortality, clinical response and radiological characteristics
BMC Pulmonary Medicine
Interstitial lung disease
Acute respiratory failure
Cyclophosphamide
Ground-glass opacification
author_facet Johanna P. van Gemert
Inge A. H. van den Berk
Esther J. Nossent
Leo M. A. Heunks
Rene E. Jonkers
Alexander P. Vlaar
Peter I. Bonta
author_sort Johanna P. van Gemert
title Cyclophosphamide for interstitial lung disease-associated acute respiratory failure: mortality, clinical response and radiological characteristics
title_short Cyclophosphamide for interstitial lung disease-associated acute respiratory failure: mortality, clinical response and radiological characteristics
title_full Cyclophosphamide for interstitial lung disease-associated acute respiratory failure: mortality, clinical response and radiological characteristics
title_fullStr Cyclophosphamide for interstitial lung disease-associated acute respiratory failure: mortality, clinical response and radiological characteristics
title_full_unstemmed Cyclophosphamide for interstitial lung disease-associated acute respiratory failure: mortality, clinical response and radiological characteristics
title_sort cyclophosphamide for interstitial lung disease-associated acute respiratory failure: mortality, clinical response and radiological characteristics
publisher BMC
series BMC Pulmonary Medicine
issn 1471-2466
publishDate 2021-07-01
description 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.
topic Interstitial lung disease
Acute respiratory failure
Cyclophosphamide
Ground-glass opacification
url https://doi.org/10.1186/s12890-021-01615-2
work_keys_str_mv AT johannapvangemert cyclophosphamideforinterstitiallungdiseaseassociatedacuterespiratoryfailuremortalityclinicalresponseandradiologicalcharacteristics
AT ingeahvandenberk cyclophosphamideforinterstitiallungdiseaseassociatedacuterespiratoryfailuremortalityclinicalresponseandradiologicalcharacteristics
AT estherjnossent cyclophosphamideforinterstitiallungdiseaseassociatedacuterespiratoryfailuremortalityclinicalresponseandradiologicalcharacteristics
AT leomaheunks cyclophosphamideforinterstitiallungdiseaseassociatedacuterespiratoryfailuremortalityclinicalresponseandradiologicalcharacteristics
AT reneejonkers cyclophosphamideforinterstitiallungdiseaseassociatedacuterespiratoryfailuremortalityclinicalresponseandradiologicalcharacteristics
AT alexanderpvlaar cyclophosphamideforinterstitiallungdiseaseassociatedacuterespiratoryfailuremortalityclinicalresponseandradiologicalcharacteristics
AT peteribonta cyclophosphamideforinterstitiallungdiseaseassociatedacuterespiratoryfailuremortalityclinicalresponseandradiologicalcharacteristics
_version_ 1721245718891462656