Abstract

BackgroundPatients with chronic known or unknown interstitial lung disease (ILD) may present with severe respiratory flares that require intensive management. Outcome data in these patients are scarce.Patients and MethodsClinical and radiological features were collected in 83 patients with ILD-associated acute respiratory failure (ARF). Determinants of hospital mortality and response to corticosteroid therapy were identified by logistic regression.ResultsHospital and 1-year mortality rates were 41% and 54% respectively. Pulmonary hypertension, computed tomography (CT) fibrosis and acute kidney injury were independently associated with mortality (odds ratio (OR) 4.55; 95% confidence interval (95%CI) (1.20–17.33); OR, 7.68; (1.78–33.22) and OR 10.60; (2.25–49.97) respectively). Response to steroids was higher in patients with shorter time from hospital admission to corticosteroid therapy. Patients with fibrosis on CT had lower response to steroids (OR, 0.03; (0.005–0.21)). In mechanically ventilated patients, overdistension induced by high PEEP settings was associated with CT fibrosis and hospital mortality.ConclusionMortality is high in ILD-associated ARF. CT and echocardiography are valuable prognostic tools. Prompt corticosteroid therapy may improve survival.

Highlights

  • Patients with chronic known or unknown interstitial lung disease (ILD) may present with severe respiratory flares that require intensive management

  • Pulmonary hypertension, computed tomography (CT) fibrosis and acute kidney injury were independently associated with mortality (odds ratio (OR) 4.55; 95% confidence interval (95%confidential intervals (CI)) (1.20–17.33); OR, 7.68; (1.78–33.22) and OR 10.60; (2.25–49.97) respectively)

  • Overdistension induced by high positive end-expiratory pressure (PEEP) settings was associated with CT fibrosis and hospital mortality

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Summary

Introduction

Patients with chronic known or unknown interstitial lung disease (ILD) may present with severe respiratory flares that require intensive management. Outcome data in these patients are scarce. One complication of ILD is acute respiratory failure (ARF), which may develop as the inaugural manifestation or as an acute exacerbation of chronic ILD. Little is known about the clinical features and outcomes of ARF complicating ILD. ARF/ARDS complicating ILD may differ from other forms of ARF/ARDS regarding the response to corticosteroids and the outcome. ILD is responsible for an increase in lung stiffness that may increase the risk of ventilator-induced injury compared to other causes of ARF or ARDS. High positive end-expiratory pressure (PEEP) was associated with increased mortality in a retrospective cohort study of patients with ILD [7]

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