Treatment Outcomes of Infectious and Non-infectious Acute Exacerbation of Myositis-Related Interstitial Lung Disease

Introduction: Although respiratory infections are common causes of acute respiratory failure (ARF) in patients with myositis-interstitial lung disease (ILD), limited data are available regarding the treatment outcomes by the etiologies of acute exacerbation (AE) of myositis-related ILD (infectious vs. non-infectious). Our study aimed to investigate the treatment outcomes of AE in patients with myositis-related ILD focused on the infectious etiology.

Methods: A single center-based retrospective cohort was performed at Hanyang University Hospital between January 2000 and December 2018. A total of 36 patients with AE of myositis-related ILD were consecutively included. The exposure was the etiologies of AE in myositis-related ILD, and the outcome was in-hospital mortality. The infectious etiology was defined as confirmation of bacteria, virus, or fungus in samples obtained from the respiratory tract.

Results: Among the 36 patients, 17 were diagnosed with infectious AE. The overall in-hospital mortality rate of AE was 47.2%. Although the mortality rate in patients with infectious AE was lower (41.2%) than in those with non-infectious AE (52.6%), this difference was not statistically significant (p = 0.724). A survival analysis showed no significant difference in mortality between patients with infectious AE versus those with non-infectious AE [risk ratio = 0.78, 95% CI = 0.38-1.59].

Conclusion: Our study showed that infectious AE is an important cause of mortality in patients with myositis-related ILD, showing a similar risk of mortality as non-infectious AE.