Abstract
BackgroundSurveys and retrospective studies of patients with idiopathic pulmonary fibrosis (IPF) have shown a significant diagnostic delay. However, the causes and risk factors for this delay are not known.MethodsDates at six time points before the IPF diagnosis (onset of symptoms, first contact to a general practitioner, first hospital contact, referral to an interstitial lung disease (ILD) centre, first visit at an ILD centre, and final diagnosis) were recorded in a multicentre cohort of 204 incident IPF patients. Based on these dates, the delay was divided into specific patient-related and healthcare-related delays. Demographic and clinical data were used to determine risk factors for a prolonged delay, using multivariate negative binomial regression analysis.ResultsThe median diagnostic delay was 2.1 years (IQR: 0.9–5.0), mainly attributable to the patients, general practitioners and community hospitals. Male sex was a risk factor for patient delay (IRR: 3.84, 95% CI: 1.17–11.36, p = 0.006) and old age was a risk factor for healthcare delay (IRR: 1.03, 95% CI: 1.01–1.06, p = 0.004). The total delay was prolonged in previous users of inhalation therapy (IRR: 1.99, 95% CI: 1.40–2.88, p < 0.0001) but not in patients with airway obstruction. Misdiagnosis of respiratory symptoms was reported by 41% of all patients.ConclusionDespite increased awareness of IPF, the diagnostic delay is still 2.1 years. Male sex, older age and treatment attempts for alternative diagnoses are risk factors for a delayed diagnosis of IPF. Efforts to reduce the diagnostic delay should focus on these risk factors.Trial registrationThis study was registered at http://clinicaltrials.gov (NCT02772549) on May 10, 2016.
Highlights
Surveys and retrospective studies of patients with idiopathic pulmonary fibrosis (IPF) have shown a significant diagnostic delay
Demographic data such as age and sex were similar in both groups, but there was a trend towards more never-smokers among patients with a long total delay (Table 1)
Patients with a total delay of more than two years were more frequently treated with inhalation therapy prior to their diagnosis but without a marked increase in airway obstruction, defined by a FEV1/FVC ratio below 0.7 (Table 1)
Summary
Surveys and retrospective studies of patients with idiopathic pulmonary fibrosis (IPF) have shown a significant diagnostic delay. Idiopathic Pulmonary Fibrosis (IPF) is a debilitating chronic lung disease. The diagnosis of IPF can be challenging, and due to its rarity, few physicians gain enough expertise to become familiar with the disease. Surveys and retrospective studies have shown a significant diagnostic delay from the onset of symptoms until the final diagnosis is made [5,6,7,8]. This results in a delayed start of effective antifibrotic treatment and lung transplant evaluation, and can affect the disease course and
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