Analysis of comorbid conditions in 1210 IPF patients from the EMPIRE registry

Jovanović, Dragana; 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; Hájková, Marta; Svoboda, Michal; Strenková, Jana; Vašáková, Martina

doi:10.1183/1393003.congress-2017.oa1953

Cited by 3 publications

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“…However, real-world evidence suggests that comorbid conditions are prevalent in patients with IPF in clinical practice, with results from the European MultiPartner IPF Registry (EMPIRE) reporting that 86% of patients with IPF had a comorbid diagnosis. 13 These data suggest that there is a need to further investigate characteristics and treatment outcomes in real-world populations of patients with IPF, which may be more representative of clinical practice compared with clinical trial populations.…”

Section: Introductionmentioning

confidence: 99%

Baseline clinical characteristics, comorbidities and prescribed medication in a real-world population of patients with idiopathic pulmonary fibrosis: the PROOF registry

et al. 2018

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IntroductionPROOF (a Prospective Observational Registry to Describe the Disease Course and Outcomes of Idiopathic Pulmonary Fibrosis) is an ongoing, observational registry initiated in 2013 with the aim of collecting real-world data from patients with idiopathic pulmonary fibrosis (IPF). Here, we present comprehensive baseline data, which were collected from patients on registry inclusion.MethodsPatients with IPF were enrolled across eight centres in Belgium and Luxembourg. Baseline data collected included demographics, diagnostic information and clinical characteristics, including lung function and health-related quality of life. Data on comorbidities and prescribed medication were also collected.ResultsA total of 277 patients were enrolled in the PROOF registry. At inclusion, 92.8% and 6.5% of patients had a definite or probable diagnosis of IPF, respectively. Mean per cent predicted forced vital capacity and carbon monoxide diffusing capacity were 80.6% and 46.9%, respectively. Mean St. George’s Respiratory Questionnaire total score was 47.0, and mean Cough-Visual Analogue Scale score was 30.5 mm. The most prevalent comorbidities reported at inclusion were gastrointestinal disorders (50.2%), including gastro-oesophageal reflux disease (47.3%) and metabolism and nutrition disorders (39.7%). At inclusion, 67.2% and 2.2% of patients were prescribed pirfenidone and nintedanib, respectively, with treatment initiated either prior to, or at the time of, inclusion. Medication prescribed concomitantly with pirfenidone included antihypertensives (54.8%), statins (37.1%) and prophylactic antithrombotics/anticoagulants (36.6%).ConclusionThe PROOF registry provides valuable demographic and clinical data from a real-world population of patients with IPF in Belgium and Luxembourg, demonstrating the high burden of comorbidities and prescribed medication in these patients. Longitudinal data from this patient population will be investigated in future analyses.Trial registrationPROOF is registered with the relevant authorities in Belgium and Luxembourg, with registration to Comité National d’Éthique et de Recherché (CNER) N201309/03 – 12 September 2013 and a notification to Comité National de Protection des Données (CNDP).

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Section: Introductionmentioning

confidence: 99%

Baseline clinical characteristics, comorbidities and prescribed medication in a real-world population of patients with idiopathic pulmonary fibrosis: the PROOF registry

et al. 2018

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“…In addition, patients with comorbid conditions, including unstable or deteriorating cardiac or pulmonary disease, or those who were receiving certain prescribed medications were excluded from trials. In contrast, evidence from real-world populations of patients with IPF suggests that there is a high burden of comorbid conditions and concomitant medication use in clinical practice [10, 11]. Rates of treatment adherence might also differ between clinical trials and real-world populations.…”

Section: Introductionmentioning

confidence: 99%

Longitudinal clinical outcomes in a real-world population of patients with idiopathic pulmonary fibrosis: the PROOF registry

et al. 2019

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BackgroundThe PROOF registry is an observational study initiated in October 2013 with the aim to monitor disease progression in a real-world population of patients with idiopathic pulmonary fibrosis (IPF). Here, we present longitudinal clinical outcomes from the PROOF registry.MethodsPatients with IPF were enrolled across eight centers in Belgium and Luxembourg. For all patients, clinical outcomes data were collected, including mortality, lung transplant, acute exacerbations, and pulmonary hypertension. For patients treated with pirfenidone at any time during follow-up (2013–2017), for any duration of treatment (the pirfenidone-treated population): pirfenidone treatment patterns were collected; changes in pulmonary function (forced vital capacity [FVC] and carbon monoxide diffusing capacity [DLco]) were reviewed up to 24 months post-inclusion; and time-to-event analyses from the time of registry inclusion were performed.ResultsThe PROOF registry enrolled a total of 277 patients. During follow-up, 23.1% of patients died, 5.1% received a lung transplant, 5.4% experienced an acute exacerbation, and 6.1% had comorbid pulmonary hypertension. In the pirfenidone-treated population (N = 233, 84.1%), 12.9% of patients had a temporary dose discontinuation and 31.8% had a temporary dose reduction; 4.3% of patients permanently discontinued pirfenidone due to an adverse drug reaction. Mean percent predicted FVC was 81.2% (standard deviation [SD] 19.0) at Month 0 and 78.3% (SD 25.0) at Month 24, and mean percent predicted DLco was 47.0% (SD 13.2) and 45.0% (SD 16.5), respectively. Rates of ≥ 10% absolute decline in percent predicted FVC and ≥ 15% absolute decline in percent predicted DLco over 24 months were 31.0% and 23.2%, respectively. Mean times from registry inclusion to categorical absolute decline in percent predicted FVC and percent predicted DLco were 20.1 (standard error [SE] 0.6) months and 23.4 (SE 0.5) months, respectively; mean time from registry inclusion to death was 31.0 (SE 0.9) months.ConclusionsThe PROOF registry is a source of European data characterizing longitudinal clinical outcomes of patients with IPF. Over 12 months of follow-up, pulmonary function remained largely stable in patients with IPF who received pirfenidone for any duration of treatment. Pulmonary function remained similar at 24 months of follow-up, although patient numbers were lower.Trial registrationPROOF is registered with the relevant authorities in Belgium and Luxembourg, with registration to Comité National d’Éthique et de Recherche (CNER) N201309/03–12 September 2013 and a notification to Comité National de Protection des Données (CNDP) for Luxembourg.

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“…In combination with the high prevalence of CVD in patients with IPF, these factors mean that cardiologists may be in a privileged position to identify patients with possible IPF and refer them to a specialist in ILD. The high burden of CVD in patients with IPF also means that cardiologists may have an important role in co-managing affected patients [10, 46]. This includes assessing patients for PH, one of the most frequent cardiovascular complications in patients with IPF [52, 54–56, 116].…”

Section: Resultsmentioning

confidence: 99%

Idiopathic Pulmonary Fibrosis for Cardiologists: Differential Diagnosis, Cardiovascular Comorbidities, and Patient Management

et al. 2018

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The presence of rare comorbidities in patients with cardiovascular disease (CVD) presents a diagnostic challenge to cardiologists. In evaluating these patients, cardiologists are faced with a unique opportunity to shorten diagnosis times and direct patients towards correct treatment pathways. Idiopathic pulmonary fibrosis (IPF), a type of interstitial lung disease (ILD), is an example of a rare disease where patients frequently demonstrate comorbid CVD. Both CVD and IPF most commonly affect a similar patient demographic: men over the age of 60 years with a history of smoking. Moreover, IPF and heart failure (HF) share a number of symptoms. As a result, patients with IPF can be misdiagnosed with HF and vice versa. This article aims to increase awareness of IPF among cardiologists, providing an overview for cardiologists on the differential diagnosis of IPF from HF, and describing the signs and symptoms that would warrant referral to a pulmonologist with expertise in ILD. Once patients with IPF have received a diagnosis, cardiologists can have an important role in managing patients who are candidates for a lung transplant or those who develop pulmonary hypertension (PH). Group 3 PH is one of the most common cardiovascular complications diagnosed in patients with IPF, its prevalence varying between reports but most often cited as between 30% and 50%. This review summarizes the current knowledge on Group 3 PH in IPF, discusses data from clinical trials assessing treatments for Group 1 PH in patients with IPF, and highlights that treatment guidelines recommend against these therapies in IPF. Finally, this article provides the cardiologist with an overview on the use of the two approved treatments for IPF, the antifibrotics pirfenidone and nintedanib, in patients with IPF and CVD comorbidities. Conversely, the impact of treatments for CVD comorbidities on patients with IPF is also discussed.Funding: F. Hoffmann-La Roche, Ltd.Plain Language Summary: Plain language summary available for this article.

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Analysis of comorbid conditions in 1210 IPF patients from the EMPIRE registry

Cited by 3 publications

References 0 publications

Baseline clinical characteristics, comorbidities and prescribed medication in a real-world population of patients with idiopathic pulmonary fibrosis: the PROOF registry

Baseline clinical characteristics, comorbidities and prescribed medication in a real-world population of patients with idiopathic pulmonary fibrosis: the PROOF registry

Longitudinal clinical outcomes in a real-world population of patients with idiopathic pulmonary fibrosis: the PROOF registry

Idiopathic Pulmonary Fibrosis for Cardiologists: Differential Diagnosis, Cardiovascular Comorbidities, and Patient Management

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