Steroid therapy in acute exacerbation of fibrotic interstitial lung disease

• Published in: Respirology (Carlton, Vic.) Vol. 29; no. 9; pp. 795 - 802
• Main Authors: Koshy, Kavya, Barnes, Hayley, Farrand, Erica, Glaspole, Ian
• Format: Journal Article
• Language: English
• Published: Chichester, UK John Wiley & Sons, Ltd 01.09.2024; Wiley Subscription Services, Inc
• Subjects: acute exacerbation; Age composition; Aged; autoimmune disease; Clinical trials; Comorbidity; Corticosteroids; Disease Progression; Female; Fibrosis; fibrotic; Glucocorticoids - therapeutic use; Hospital Mortality; Humans; Idiopathic Pulmonary Fibrosis - drug therapy; Idiopathic Pulmonary Fibrosis - mortality; interstitial lung disease; Lung diseases; Lung Diseases, Interstitial - drug therapy; Lung Diseases, Interstitial - mortality; Lung Diseases, Interstitial - physiopathology; Lung Transplantation; Male; Middle Aged; Mortality; Patients; Prednisolone; Prednisolone - therapeutic use; pulmonary fibrosis; Retrospective Studies; Steroid hormones; steroid therapy; Steroids; Survival; Treatment Outcome; acute exacerbation; fibrotic; steroid therapy; interstitial lung disease; autoimmune disease; pulmonary fibrosis
• ISSN: 1323-7799; 1440-1843; 1440-1843
• DOI: 10.1111/resp.14763

Background and Objective Evidence for the benefit of steroid therapy in acute exacerbations (AEs) of idiopathic pulmonary fibrosis (IPF) is limited; however, they remain a cornerstone of management in other fibrotic interstitial lung diseases. This retrospective observational study assesses the effect of steroid treatment on in‐hospital mortality in patients with acute exacerbation of fibrotic interstitial lung disease (AE‐FILD) including IPF and non‐IPF ILDs. Methods AE‐FILD cases over a 10‐year period were filtered using a code‐based algorithm followed by individual case evaluation. Binary logistic regression analysis was used to assess the relationship between corticosteroid treatment (defined as ≥0.5 mg/kg/day of prednisolone‐equivalent for ≥3 days within the first 72 h of admission) and in‐hospital mortality or need for lung transplantation. Secondary outcomes included readmission, overall survival, requirement for domiciliary oxygen and rehabilitation. Results Across two centres a total of 107 AE‐FILD subjects were included, of which 46 patients (43%) received acute steroid treatment. The steroid cohort was of younger age with fewer comorbidities but had higher oxygen requirements. Pre‐admission FVC and DLCO, distribution of diagnoses and smoking history were similar. The mean steroid treatment dose was 4.59 mg/kg/day. Steroid use appeared to be associated with increased risk of inpatient mortality or transplantation (OR 4.11; 95% CI 1.00–16.83; p = 0.049). In the steroid group, there appeared to be a reduced risk of all‐cause mortality in non‐IPF patients (HR 0.21; 95% CI 0.04–0.96; p = 0.04) compared to their IPF counterparts. Median survival was reduced in the steroid group (221 vs. 520.5 days) with increased risk of all‐cause mortality (HR 3.25; 95% CI 1.56–6.77; p < 0.01). Conclusion In this two‐centre retrospective study of 107 patients, AE‐FILD demonstrates a high risk of mortality, at a level similar to that seen for AE‐IPF, despite steroid treatment. Clinicians should consider other precipitating factors for exacerbations and use steroids judiciously. Further prospective trials are needed to determine the role of corticosteroids in AE‐FILD. This is a multicentre retrospective observational study comparing the outcomes of acute exacerbation of fibrotic ILD between steroid and non‐steroid treatment. Those who were administered steroids were younger, had fewer comorbidities but greater oxygen requirement and lower physiologic reserve. When adjusted for these variables, there appeared no survival benefit and potential increased risk of death in those who were given steroids. See related editorial