Abstract

Interstitial lung disease (ILD), particularly idiopathic pulmonary fibrosis (IPF), has a poor prognosis. Corticosteroids are widely used in the treatment of acute exacerbation of ILD (AE-ILD). This study aimed to clarify the causes of AE-ILD, determine the efficacy of corticosteroids for treating AE-ILD, and detect differences in the mortality rate among subgroups of ILD. This was an observational retrospective single-center study. Patients with ILD who presented to the emergency department with acute respiratory symptoms from January 1, 2016, to December 31, 2018, were included. Patients with AE-ILD were classified into two groups depending on the prednisolone dose: low dose (0 to 1.0 mg/kg) or high dose (> 1.0 mg/kg). Mortality rates between patients with and without IPF were compared. This study included 182 patients with AE-ILD, including IPF (n = 117) and non-IPF (n = 65). Multivariate Cox regression analysis showed that corticosteroid dose (HR: 0.221, CI: 0.102–0.408, P < 0.001), initial P/F ratio (HR:0.995, CI:0.992–0.999, P = 0.006), and mechanical ventilation within 3 days of hospitalization (HR:4.205, CI:2.059–8.589, P < 0.001) were independent risk factors for mortality in patients with AE-ILD. This study showed that outcomes improve with higher doses of corticosteroids (> 1 mg/kg prednisolone) in patients with AE-non-IPF-ILD. However, this was not the case in patients with AE-IPF.

Highlights

  • Interstitial lung disease (ILD), idiopathic pulmonary fibrosis (IPF), has a poor prognosis

  • We reviewed medical records and all chest computed tomography (CT) scans of patients who were diagnosed with ILD and presented to the emergency department (ED) with symptoms of dyspnea at the Severance Hospital between January 2016 and December 2018

  • In June 2019, all subjects were reevaluated by multidisciplinary discussion using patient history, physical examination, laboratory results, chest high-resolution CT (HRCT), and histopathological findings

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Summary

Introduction

Interstitial lung disease (ILD), idiopathic pulmonary fibrosis (IPF), has a poor prognosis. This study aimed to clarify the causes of AE-ILD, determine the efficacy of corticosteroids for treating AE-ILD, and detect differences in the mortality rate among subgroups of ILD. This study showed that outcomes improve with higher doses of corticosteroids (> 1 mg/kg prednisolone) in patients with AE-non-IPF-ILD. This was not the case in patients with AE-IPF. High-dose corticosteroids, sometimes administered as pulse therapy (with or without immunosuppressive agents) in combination with broad-spectrum antimicrobial agents, have been used most commonly in patients with AE-ILD This is the recommended treatment according to recent international treatment guidelines for ­IPF12. The efficacy of this therapy is uncertain, and survival is still very ­low[4]

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