A new study reported that patients with newly diagnosed idiopathic pulmonary fibrosis (IPF) often experience lung function decline, which furthers disease progression. The study, titled “Change in forced vital capacity and associated subsequent outcomes in patients with newly diagnosed idiopathic pulmonary fibrosis,” was published in the journal BMC Pulmonary Medicine.
Despite its unknown etiology, IPF is a severe disease characterized by progressive loss of lung function and frequently culminating in patients’ death (median survival time is approximately 3–5 years after diagnosis). IPF patients can present a variable clinical course, with some patients remaining stable, and others progressing steadily or rapidly experiencing episodes of acute exacerbations. This variability poses a challenge in predicting disease progression and consequently managing patients’ care.
A further challenge is the lack of studies investigating how alterations in lung function impact clinical outcomes, including acute exacerbations and healthcare resource utilization (HRU) in patients.
The research team used a new approach to tackle this unanswered question, conducting a retrospective chart review of patients diagnosed with IPF though a panel of over 1,000 pulmonologists from all regions in the United States. IPF patients included in the study were at least 40 years old and had a confirmed diagnosis of IPF by high-resolution computed tomography and/or lung biopsy.
In total, 490 IPF patients were analyzed (68% males), with a mean age of 61. Changes in forced vital capacity (FVC), a measure of lung function, were registered both at diagnosis and six months after, and correlated with the patients’ clinical and HRU outcomes.
The team found that approximately half of the IPF patients analyzed exhibited FVC decline within six months of diagnosis. Moreover, it observed that following diagnosis, clinical characteristics and HRU were similar between groups with different FVCs; however, escalating decline in FVC percentage was associated with worse clinical outcomes and increased HRU in the follow-up period. The results suggest that greater lung function decline is associated with an increased risk of further IPF progression, including acute exacerbations, HRU, and ultimately higher mortality rates.
In conclusion, the findings support measurements that prevent or ameliorate lung function deterioration (here determined by decline in FVC) as a strategy to improve health outcomes in patients with IPF.