A new study highlights the lengthy delay in diagnosis of idiopathic pulmonary fibrosis (IPF) and the need for optimization of disease management.

The study reached out to 5193 physicians across France, Germany, Japan, and the US as part of the Adelphi IPF II Disease Specific Programme. A total of 244 physicians reported information on 1249 patients with IPF, and 739 of those patients subsequently provided self-reported data. The patients in the study were mostly male (mean 67.8%) and a large proportion were previous smokers (40%-68%).

The average time from first symptoms to IPF diagnosis ranged from 0.8 years to 2.0 years depending on the country. The median time between symptom onset and seeking medical assistance was 6 months (range, 0-60) while it took 7 months (range, 0-90) between first visit and diagnosis, across all patients. After diagnosis, the time until first treatment ranged from roughly 0.3 years to >1 year, depending on the country.

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“In conclusion, there is substantial need to optimize the current management approach for IPF given the rapidly progressive and irreversible nature of IPF, the considerable diagnostic delays that persist and the resulting disease burden,” the authors said.

During the diagnostic process, 7-10 different clinical tests were performed per patient. The most commonly prescribed tests were high-resolution computed tomography (80%) and pulmonary function tests (73%).

Physicians reported that 75% of patients had shortness of breath before or at the time of diagnosis. The study found that up to 45% of early-stage patients may have been misdiagnosed with chronic obstructive pulmonary disease (COPD), highlighting the need for better diagnostic testing.

Patients with severe IPF in all countries surveyed indicated that the disease had a major negative impact on their daily lives, including their social life with relatives and friends. Based on the results of the EuroQol-5 Dimensions questionnaire, patients with moderate IPF had values worse than those with moderate asthma and equal to or worse than moderate COPD. In IPF patients who were not yet retired, 28%-45% reported that IPF impaired their ability to work.

The study also found that the use of antifibrotics in IPF patients has increased over the years. In 2019, 69% of patients received antifibrotic treatment compared to 57% in 2016. Only 13%-41% of severe IPF patients and 47%-61% of moderate IPF patients were satisfied with their current treatments.

“Efforts are required to reduce diagnostic uncertainty to ensure that patients receive earlier intervention, and to improve treatment satisfaction in terms of impact on symptoms, safety and emotional well-being and QoL,” the authors added.


Lancaster L, Bonella F, Inoue Y, et al. Idiopathic pulmonary fibrosis: physician and patient perspectives on the pathway to care from symptom recognition to diagnosis and disease burden. Respirology. Published online October 5, 2021. doi:10.1111/resp.14154