Researchers discovered that patients with acute exacerbations of idiopathic pulmonary fibrosis (IPF) have a high inspiratory effort that is partially relieved by noninvasive ventilation, according to a study published in Pulmonology. 

IPF is a rare disease characterized by progressive deterioration of pulmonary function. The prognosis is exceedingly poor; average survival is around 5 years from diagnosis. In addition to progressive lung failure, patients with IPF may experience recurring acute exacerbation episodes that can lead to severe hypoxemia. 

“During these events, the typical usual interstitial pneumonia pattern—the radiological and histological hallmark of IPF—overlaps with diffuse alveolar damage sharing similarities with ARDS and often requiring respiratory support,” the authors wrote. 

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There is a gap in the medical literature regarding lung mechanics in patients with IPF during an acute exacerbation episode; hence, the researchers set out to investigate how lung mechanics under these conditions compare with those in ARDS patients of similar severity. 

Read more about IPF prognosis 

The research team retrospectively enrolled patients with IPF who have developed an acute exacerbation and were hospitalized as a result. Enrolled patients were matched to a group of patients with ARDS under spontaneous breathing according to age, sequential organ failure assessment score, PaO2/FiO2 level, and body mass index. The primary outcome measured was esophageal pressure swing and respiratory mechanics before and after noninvasive ventilation. 

The researchers discovered that patients with acute exacerbations of IPF have a notable increase in inspiratory effort, reflecting a greater activation of the respiratory drive during unassisted breathing when compared with patients with ARDS. In addition, they demonstrated lower effort and respiratory frequency under short-term noninvasive ventilation; however, no positive changes in dynamic compliance was observed. 

“In this physiologic, preliminary retrospective study, spontaneously breathing patients with IPF showed an elevated inspiratory effort while on acute exacerbation of the disease,” the authors concluded. 


Tonelli R, Castaniere I, Cortegiani A, et al. Inspiratory effort and respiratory mechanics in patients with acute exacerbation of idiopathic pulmonary fibrosis: a preliminary matched control studyPulmonology. 2022;S2531-0437(22)00204-5. doi:10.1016/j.pulmoe.2022.08.004