Does timeliness of diagnosis influence survival and treatment response in idiopathic pulmonary fibrosis? Real- world results from the EMPIRE registry

Vašáková, Martina; Moğulkoç, Nesrin; Šterclová, Martina; Zolnowska, Beata; Bartoš, Vladimı́r; Plačková, Martina; Müller, Veronika; Lacina, Ladislav; Slivka, Róbert; Doubková, Martina; Milenkovic, Marina Roksandić; Bittenglová, Radka; Jonner, Imrich; Martusewicz-Boros, Magdalena; Žůrková, Monika; Binková, Ilona; Sobiecka, Małgorzata; Tyl, Richard; Trzaska-Sobczak, Marzena; Śliwiński, Paweł; Lošťáková, Vladimíra; Majewski, Sebastian; Lisá, Pavlína; Lewandowska, Katarzyna; Šuldová, Hana; Tekavec-Trkanjec, Jasna; Szymanowska-Narloch, Amelia; Šišková, Lenka; Bohács, Anikó; Plutinský, Ján; Matula, Bohumil; Tóth, Štefan; Petřík, František; Bučeková, Margita; Pšíkalová, Jana; Zsiray, Miklós; Lněnička, Jaroslav; Řihák, Vladimír; Szalai, Zsuzsanna; Balikó, Zoltán; Laššán, Štefan; Musilova, Pavlina; Lajkó, Imre; Snížek, Tomáš; Anton, Jan; Homolka, J; Paluch, Peter; Vyšehradský, Róbert; Králová, Renata; Kervitzer, Jan; Doležal, Daniel; Szilasi, Mária; Kuś, Jan; Jovanović, Dragana; Hájková, Marta; Svoboda, Michal; Strenková, Jana

doi:10.1183/1393003.congress-2017.pa4880

Cited by 8 publications

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“…Reported time to diagnosis from the onset of initial symptoms varies in different studies but may be up to a median of 2.1 years (IQR 0.9-5.0) (5). Longer time to diagnosis is associated with worse outcomes in IPF (6,7), causes delayed treatment, leads to more extensive fibrosis (8) and affects patients' well-being. Therefore, it is important to get better insights into patients' experiences during the diagnostic journey to identify reasons for potential delays.…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2021

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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.

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Section: Introductionmentioning

confidence: 99%

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

et al. 2021

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“…A major unmet need in IPF care is the provision of timely and accurate diagnosis [ 13 , 18 , 22 ] and this is a recurrent deficit in health service provision. Several studies have highlighted the scope of the problem, with many patients diagnosed with IPF experiencing a protracted route to diagnosis [ 7 , 18 , 20 , 22 , 41 , 43 , 44 , 52 ] which leads to unnecessary delays including accessing pharmacological interventions and other supportive treatments [ 79 ].…”

Section: Resultsmentioning

confidence: 99%

“…[ 20 , 81 ] Internationally and across several healthcare systems there remain deficits in providing patients with an early and accurate diagnosis [ 13 , 18 , 22 ], representing a recurrent gap in health service provision. These delays have significant impacts to patients, including accessing pharmacological interventions and other supportive treatments [ 79 ].…”

Section: Discussionmentioning

confidence: 99%

A scoping review of the unmet needs of patients diagnosed with idiopathic pulmonary fibrosis (IPF)

Bramhill,

Langan,

Mulryan

et al. 2024

PLoS ONE

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Aims Patients diagnosed with idiopathic pulmonary fibrosis (IPF) have a high symptom burden and numerous needs that remain largely unaddressed despite advances in available treatment options. There is a need to comprehensively identify patients’ needs and create opportunities to address them. This scoping review aimed to synthesise the available evidence and identify gaps in the literature regarding the unmet needs of patients diagnosed with IPF. Methods The protocol for the review was registered with Open Science Framework (DOI 10.17605/OSF.IO/SY4KM). A systematic search was performed in March 2022, in CINAHL, MEDLINE, Embase, PsychInfo, Web of Science Core Collection and ASSIA Applied Social Science Index. A comprehensive review of grey literature was also completed. Inclusion criteria included patients diagnosed with IPF and date range 2011–2022. A range of review types were included. Data was extracted using a data extraction form. Data was analysed using descriptive and thematic analysis. A total of 884 citations were reviewed. Ethical approval was not required. Results 52 citations were selected for final inclusion. Five themes were identified: 1.) psychological impact of an IPF diagnosis. 2.) adequate information and education: at the right time and in the right way. 3.) high symptom burden support needs. 4.) referral to palliative care and advance care planning (ACP). 5.) health service provision-a systems approach. Conclusion This review highlights the myriad of needs patients with IPF have and highlights the urgent need for a systems approach to care, underpinned by an appropriately resourced multi-disciplinary team. The range of needs experienced by patients with IPF are broad and varied and require a holistic approach to care including targeted research, coupled with the continuing development of patient-focused services and establishment of clinical care programmes.

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“…Several studies have highlighted the scope of the problem with many patients diagnosed with IPF, experiencing a protracted route to diagnosis (17, 34) (6, 16, 35, 44) (19, 36) leading to unnecessary delays including accessing pharmacological interventions and other supportive treatments. (74)…”

Section: Resultsmentioning

confidence: 99%

A scoping review of the unmet needs of patients diagnosed with idiopathic pulmonary fibrosis (IPF)

Bramhill,

Langan,

Mulryan

et al. 2023

Preprint

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TitleA scoping review of the unmet needs of patients diagnosed with idiopathic pulmonary fibrosis (IPF).AimsPatients diagnosed with IPF have a high symptom burden and numerous needs that remain largely unaddressed despite advances in available treatment options. There is a need to comprehensively identify patients’ needs and create opportunities to address them. This scoping review aimed to synthesise the available evidence and identify gaps in the literature regarding the unmet needs of patients diagnosed with IPF.MethodsThe protocol for the review was registered with Open Science Framework (DOI 10.17605/OSF.IO/SY4KM). A systematic search was performed in March 2022, in CINAHL, MEDLINE, Embase, PsyhcoInfo, Web of Science Core Collection and ASSIA Applied Social Science Index. A comprehensive review of grey literature was also completed. Inclusion criteria included patients diagnosed with IPF or PF; English language only and date range 2011-2022. A range of review types were included. Data was extracted using a data extraction form. Data was analysed using descriptive and thematic analysis. A total of 884 citations were reviewed. Ethical approval was not required.Results52 citations were selected for final inclusion. Five themes were identified: psychological impact of an IPF diagnosis; adequate information and education: at the right time and in the right way; high symptom burden support needs; referral to palliative care and advanced care planning (ACP) and health service provision-a systems approach.ConclusionThis review highlights the myriad of needs patients with IPF have and highlights the urgent need for a systems approach to care, underpinned by an appropriately resourced multi-disciplinary team. The range of needs experienced by patients with IPF are broad and varied and require a holistic approach to care including targeted research, coupled with the continuing development of patient-focused services and development of a clinical care programme.

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Does timeliness of diagnosis influence survival and treatment response in idiopathic pulmonary fibrosis? Real- world results from the EMPIRE registry

Cited by 8 publications

References 0 publications

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

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

A scoping review of the unmet needs of patients diagnosed with idiopathic pulmonary fibrosis (IPF)

A scoping review of the unmet needs of patients diagnosed with idiopathic pulmonary fibrosis (IPF)

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