Clinical outcomes of patients with acute exacerbations in interstitial lung diseases

Abstract

Introduction: Acute exacerbations in interstitial lung diseases (AE-ILD) have been defined as an acute, clinically significant deterioration that develops within less than one month without an obvious clinical cause. It has poor prognosis and is associated with a high mortality within 6-12 months.

Aim: To evaluate clinical outcomes of patients with AE-ILD.

Methods: Single-center retrospective study including adult patients admitted due to AE-ILD from november 2007 to december 2021. Clinical and lung function (LF) data before and after AE-ILD were collected.

Results: A total of 59 patients (median age 70.0±10.08 years, 69.5% male) were admitted to AE-ILD and an inaugural ILD diagnosis was established in 7 of these patients. Hypersensitivity pneumonitis (HP) was the most frequent diagnosis (n=27, 45.8%) followed by idiopathic pulmonary fibrosis (IPF) in 37.3% of the cases. Due to respiratory failure, non-invasive ventilation (NIV) was used in 54.2% (n=32) of the cases and high flow nasal oxygen in 26 patients. Antibiotic therapy and methylprednisolone pulses were initiated in 84.7% and 93.2% of the cases, respectively. Other immunosuppressive therapy was implemented in 14 patients. Most of the patients died (n=32, 54.2%) and these patients displayed a lower DLCO (2.56±0.98, p=0.033). The use of NIV showed a tendency to higher mortality (p=0.056). There was no significant difference in mortality between HP and IPF (p=0.278). Following hospital discharge, it was verified a 6th month mortality of 63% (n=17).

Conclusions: HP was the most common ILD with AE. AE-ILD is associated with high mortality during hospitalization and at 6 months. Lower DLCO in recent LF tests was associated with higher mortality.