Patient Reported Experiences and Delays During the Diagnostic Pathway for Pulmonary Fibrosis: A Multinational European Survey

Abstract

Introduction: Pulmonary fibrosis includes a spectrum of diseases and is incurable. There is a variation in disease course, but it is often progressive leading to increased breathlessness, impaired quality of life, and decreased life expectancy. Detection of pulmonary fibrosis is challenging, which contributes to considerable delays in diagnosis and treatment. More knowledge about the diagnostic journey from patients' perspective is needed to improve the diagnostic pathway. The aims of this study were to evaluate the time to diagnosis of pulmonary fibrosis, identify potential reasons for delays, and document patients emotions.Methods: Members of European patient organisations, with a self-reported diagnosis of pulmonary fibrosis, were invited to participate in an online survey. The survey assessed the diagnostic pathway retrospectively, focusing on four stages: (1) time from initial symptoms to first appointment in primary care; (2) time to hospital referral; (3) time to first hospital appointment; (4) time to final diagnosis. It comprised open-ended and closed questions focusing on time to diagnosis, factors contributing to delays, diagnostic tests, patient emotions, and information provision.Results: Two hundred and seventy three participants (214 idiopathic pulmonary fibrosis, 28 sarcoidosis, 31 other) from 13 countries responded. Forty percent of individuals took ≥1 year to receive a final diagnosis. Greatest delays were reported in stage 1, with only 50.2% making an appointment within 3 months. For stage 2, 73.3% reported a hospital referral within three primary care visits. However, 9.9% reported six or more visits. After referral, 76.9% of patients were assessed by a specialist within 3 months (stage 3) and 62.6% received a final diagnosis within 3 months of their first hospital visit (stage 4). Emotions during the journey were overall negative. A major need for more information and support during and after the diagnostic process was identified.Conclusion: The time to diagnose pulmonary fibrosis varies widely across Europe. Delays occur at each stage of the diagnostic pathway. Raising awareness about pulmonary fibrosis amongst the general population and healthcare workers is essential to shorten the time to diagnosis. Furthermore, there remains a need to provide patients with sufficient information and support at all stages of their diagnostic journey.

Exploration of the unmet needs of patients diagnosed with idiopathic pulmonary fibrosis: a scoping review protocol

“…Most types of PF are identified by a progressive phenotype, characterised by breathlessness, cough and fatigue. 3 4 The most common type of PF is idiopathic pulmonary fibrosis (IPF), accounting for around 17%–37% of all ILDs. 5 IPF is a chronic progressive disease, carrying with it potentially significant impacts on both physical and emotional well-being.…”

An exploration of the unmet needs of patients diagnosed with Idiopathic Pulmonary Fibrosis (IPF): a scoping review protocol

“…A large subset of patients who are diagnosed with ILD have pulmonary fibrosis (PF). Most types of pulmonary fibrosis are identified by a progressive phenotype characterised by breathlessness, cough and fatigue 1 6 . The most common PF is IPF, and accounts for around 17-37% of all ILDs 7 .…”

“…The literature widely reports continuing delays in’ early diagnosis and referrals to specialist centres 6 10 . According to Van der Sar 6 some of the delays relating to diagnosis relate to the heterogeneity of the disease, rarity of pulmonary fibrosis as well as the requirements for multiple investigations resulting in a prolonged delay in diagnosis 43 .…”

Early Diagnosis of Interstitial Lung Disease

“…In addition, it can also be caused by a misdiagnosis by clinicians in the community so that they are late in referring to a tertiary health centre. Therefore, it is hoped that primary health services and pulmonary doctors can consider the diagnosis of ILD in patients with symptoms of shortness of breath of unknown cause and then involve other expertise in diagnosing and managing ILD early in the course of the disease 16,19. Currently, screening for ILD is limited to patients with known risk factors for ILD or a history of familial IPF.…”

“…All of these contribute to the delay in the diagnosis of ILD. Research on biomarkers and quantitative imaging methods currently being developed can be the key to identifying ILD as early as possible 16,19. Screening for ILD in patients with systemic sclerosis with HRCT shows that 60% of whom has normal lung function.…”