Demographic and clinical profile of idiopathic pulmonary fibrosis patients in Spain: the SEPAR National Registry

Fernández-Fabrellas, Estrella; Molina-Molina, María; Soriano, Joan B.; Portal, José Antonio Rodríguez; Ancochea, Julio; Valenzuela, Claudia; Xaubet, Antoni

doi:10.1186/s12931-019-1084-0

Cited by 38 publications

(42 citation statements)

References 45 publications

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“…Approximately 70% of patients were receiving an antifibrotic drug at enrollment in the IPF-PRO Registry. This is similar to the proportions of patients with IPF who were treated with antifibrotic medication at enrollment in other registries in the United States ( 14 ), Europe ( 15 – 17 ), and Latin America ( 20 ), although some reports from Europe document lower use ( 18 , 19 ). Variation in the proportions of patients treated with antifibrotic medications across these registries could relate to differences in healthcare systems and access to treatment, the types of site at which patients were recruited, the timing of data collection, and the methodology used to recruit patients and analyze treatment status.…”

Section: Discussionsupporting

confidence: 82%

Antifibrotic Drug Use in Patients with Idiopathic Pulmonary Fibrosis. Data from the IPF-PRO Registry

et al. 2020

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Rationale: Two antifibrotic medications, nintedanib and pirfenidone, have been approved for the treatment of idiopathic pulmonary fibrosis (IPF) in the United States. Few data have been published on the use of these medications in clinical practice. Objectives: To investigate patterns of use of antifibrotic medications in the United States. Methods: The Idiopathic Pulmonary Fibrosis Prospective Outcomes (IPF-PRO) Registry, a multicenter U.S. registry, has enrolled patients with IPF that was diagnosed or confirmed at the enrolling center in the past 6 months. Data from patients enrolled from June 5, 2014, to March 4, 2018, were used to determine antifibrotic medication use (“treatment”) in the enrollment window and in a follow-up window approximately 6 months later. Associations between patient characteristics and treatment status were tested using logistic regression. Results: Overall, 551 of 782 eligible patients (70.5%) were treated in the enrollment window. Younger age, lower forced vital capacity percentage predicted, oxygen use with activity, worse self-rated health (based on the Short Form 12 or St. George’s Respiratory Questionnaire score), referral to the enrolling center by a pulmonologist, use of a lung biopsy in diagnosis, and carrying a diagnosis of IPF to the enrolling center were associated with being treated. Among 534 patients treated at enrollment who had follow-up data, 94.0% remained treated in follow-up. Better self-rated health (based on the Short Form 12 mental component score or EuroQoL score) and not using oxygen with activity at enrollment were associated with continuing treatment in follow-up. Among 172 patients who were untreated at enrollment and had follow-up data, 29.7% started treatment in follow-up. Lower diffusing capacity of the lung for carbon monoxide percentage predicted, a family history of interstitial lung disease, a history of sleep apnea, and a definite diagnosis of IPF at enrollment were associated with starting treatment in follow-up. Conclusions: The majority of patients in the IPF-PRO Registry were receiving an approved medication for IPF at enrollment. Treatment at enrollment was associated with greater disease severity, more compromised quality of life, and the use of oxygen with activity. Clinical trial registered with ClinicalTrials.gov (NCT01915511).

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Section: Discussionsupporting

confidence: 82%

Antifibrotic Drug Use in Patients with Idiopathic Pulmonary Fibrosis. Data from the IPF-PRO Registry

et al. 2020

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“…The baseline lung function is analogous to that reported in the AIPFR(5), PROOF(22), SEPAR (26) and EMPIRE (24) registries as well as the INPULSIS study (4). Although the gas transfer measurement was comparable, the mean FVC reported in our Registry was higher than that from the eurIPFreg(23), IPF-PRO (19) and ASCEND study (3).…”

Section: Discussionsupporting

confidence: 80%

“…Our baseline demographics are similar to other reported registries (5,19,(21)(22)(23)(24)(25)(26) as well as the inclusion criteria for the ASCEND(3) and INPULSIS(4) clinical trials with patients being predominantly male, over the age of 60 years and ex-smokers. However, our mean (SD) age at enrolment is higher than that reported by a number of worldwide registries (67-71±8 years) (5,(19)(20)(21)(22)(23)(24)(25) but comparable to the FinnishIPF Registry (73±9.0 years) (27).…”

Section: Discussionsupporting

confidence: 73%

Idiopathic pulmonary fibrosis in the UK: analysis of the British Thoracic Society electronic registry between 2013 and 2019

et al. 2020

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Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and terminal interstitial lung disease (ILD) with a median survival of 3–5 years. The British Thoracic Society (BTS) established the UK IPF Registry in 2013 as a platform to collect data on clinical characteristics, treatments and outcomes for this cohort in the UK.Between 1st January 2013 and 31st October 2019, 2474 cases were registered. Most patients were male (79%) with a mean (sd) age of 74±8.3 and 66% were ex-smokers. Over time we observed an increase in the number of patients aged over 70. However, we have not seen a trend towards earlier presentation as symptoms of breathless and/or cough were present for more than 12 months in 63% of the cohort. At presentation, mean (sd) percent predicted FVC was 78.2±18.3, median 76.2 (IQR 65.888.2) and TLco 48.4±16.0, median 47.5 (IQR 37.3, 57.4). Most cases were discussed at an ILD multi-disciplinary meeting, with an increase over this time in the number of cases reported as having possible UIP pattern on HRCT thorax. We noted a reduction in the number of patients undergoing surgical lung biopsy or bronchoalveolar lavage. Although more patients were prescribed anti-fibrotic therapies from 2013 to 2019, 43% were ineligible for treatment based upon NICE prescribing criteria. Hypertension, ischaemic heart disease, diabetes mellitus and gastro-oesophageal reflux were the most common co-morbidities.In conclusion, we have presented baseline demographics as well as diagnostic and treatment strategies from the largest single-country IPF registry, reflecting changes in UK practices over this period.

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“…Nine of 40 deaths (22.5%) in the mild group occurred due to lung cancer, signi cantly more than those in the moderate and severe groups. The two studies mentioned above did not report the number of deaths due to lung cancer, though mortality rate due to lung cancer in the SEPAR National Registry was reported as 9.1% [16] and in a Hokkaido study as approximately 11% [17]. Despite the considerable number of deaths due to unknown causes in our study, deaths due to lung cancer constituted a signi cant proportion of deaths in the mild group (22.5%), which probably could have affected the survival prognosis.…”

Section: Discussionmentioning

confidence: 99%

Effect of antifibrotic agents on survival in idiopathic pulmonary fibrosis patients according to forced vital capacity: a real-world retrospective cohort study in Japan

Saeki¹,

Nishiyama²,

Yamazaki³

et al. 2020

Preprint

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Background Antifibrotic agents suppress the decline in forced vital capacity (FVC) and disease progression in idiopathic pulmonary fibrosis (IPF) patients. However, their effect on survival prognosis and differences in this effect according to baseline lung function have been unexplored. Therefore, this study aimed to examine the effect of antifibrotics on survival prognosis and whether this effect differed according to baseline FVC. Methods Consecutive IPF patients from January 2008 to May 2019 were examined retrospectively. FVC and effect of pirfenidone or nintedanib therapy were assessed. FVC at registration was used to categorize the patients into mild: FVC % predicted ≥ 80%, moderate: FVC % predicted 50–80%, and severe: FVC % predicted < 50% IPF groups. Results In total, 172 IPF patients were included. The mean FVC % predicted was 77.4 ± 22.2%. The median survival periods of patients in the mild, moderate, and severe IPF groups were 1,452, 1,305, and 481 days, respectively. Significant differences were observed in survival between the mild and severe groups and the moderate and severe groups (p < 0.0001), but not between the mild and moderate groups (p = 0.20). The survival was longer in patients on antifibrotic therapy in the mild (p = 0.18) and moderate groups (p = 0.04), but not in the severe group (p = 0.93). Conclusions Antifibrotics extended the survival of IPF patients. The effect was obvious in patients with FVC % predicted of 50–80%, a tendency was observed in patients with FVC % predicted ≥ 80%, while no effect was observed in patients with FVC % predicted < 50%.

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Demographic and clinical profile of idiopathic pulmonary fibrosis patients in Spain: the SEPAR National Registry

Cited by 38 publications

References 45 publications

Antifibrotic Drug Use in Patients with Idiopathic Pulmonary Fibrosis. Data from the IPF-PRO Registry

Antifibrotic Drug Use in Patients with Idiopathic Pulmonary Fibrosis. Data from the IPF-PRO Registry

Idiopathic pulmonary fibrosis in the UK: analysis of the British Thoracic Society electronic registry between 2013 and 2019

Effect of antifibrotic agents on survival in idiopathic pulmonary fibrosis patients according to forced vital capacity: a real-world retrospective cohort study in Japan

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