Guidelines for the Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis

Xaubet, Antoni; Ancochea, Julio; Bollo, Elena; Fernández-Fabrellas, Estrella; Franquet, Tomás; Molina-Molina, María; Montero, M. Ángeles; Serrano-Mollar, Anna

doi:10.1016/j.arbr.2013.06.003

Cited by 17 publications

(24 citation statements)

References 76 publications

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“…Pirfenidone is indicated in adults for the treatment of mild-tomoderate IPF (FVC > 50% predicted [5][6][7][8][9]. The NICE recommends pirfenidone in patients with FVC between 50 and 80% predicted [10].…”

Section: Posology and Methods Of Administrationmentioning

confidence: 99%

“…In Phase III randomized clinical trials with interferon gamma 1-b, pirfenidone, macitentan, bosentan, ambrisentan, warfarin, triple therapy (n-acetylcisteine, glucocorticoids, azathitoprine), etanercept and sildenafil, only pirfenidone (Esbriet) has shown any beneficial effects in patients with IPF (Box 1) [2][3][4]. Several national guidelines on IPF diagnosis and treatment from Spain, Germany, Denmark, Sweden, Austria and Ireland have recently recommended pirfenidone as first-choice therapeutic agent in IPF patients with mild-to-moderate disease, defined as forced vital capacity (FVC) predicted > 50% [5][6][7][8][9]. In some other European countries, such as Italy, pirfenidone is, however, already commercially available.…”

Section: Introductionmentioning

confidence: 99%

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Pirfenidone for the treatment of idiopathic pulmonary fibrosis

Xaubet

Serrano-Mollar

Ancochea

2013

Expert Opinion on Pharmacotherapy

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Pirfenidone is the only anti-fibrotic drug approved for the treatment of IPF. Pirfenidone provides a meaningful clinical effect on reductions in the decrease in forced vital capacity (FVC), six-minute walk test (6MWT) distance and mortality, and it improves the progression-free survival in IPF patients with mild-to-moderate disease. Pirfenidone is well tolerated, with the most common side-effects being gastrointestinal discomfort and photosensitivity. Pirfenidone has a favorable benefit-risk profile and represents a suitable treatment option for patients with mild-to-moderate IPF.

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Section: Posology and Methods Of Administrationmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Pirfenidone for the treatment of idiopathic pulmonary fibrosis

Xaubet

Serrano-Mollar

Ancochea

2013

Expert Opinion on Pharmacotherapy

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“…Such work will be valuable to those making decisions about resource use and allocation for patients with IPF. Such work will also help inform and guide future efforts at assessing and comparing treatments for the disease, which is critical given the limited range of drugs and alternative treatments available, including lung transplantation, oxygen therapy, and pulmonary rehabilitation [2,10].…”

Section: Discussionmentioning

confidence: 99%

“…Nintedanib is a specific tyrosine kinase inhibitor, also targeting PDGF, FGF and VEGF receptors. Nintedanib was shown to inhibit fibroblast proliferation, migration, contraction and differentiation to the active extracellular matrix-secreting myofibroblast [9,10]. Nintedanib has also demonstrated to have anti-inflammatory activities by reducing inflammatory mediators IL-1b and IL-6.…”

Section: Introductionmentioning

confidence: 99%

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Influence of Idiopathic Pulmonary Fibrosis Progression on Healthcare Resource Use

Διαμαντόπουλος¹,

Maher

Schoof

et al. 2018

PharmacoEconomics Open

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Background Disease progression and acute exacerbations in patients with idiopathic pulmonary fibrosis (IPF) are associated with high morbidity and mortality. They usually require a visit to a specialist or a general practitioner (GP) in less severe cases or hospitalisation in more severe cases. Objective The objective of this study was to identify factors that influence resource use in IPF. Methods Clinical and healthcare resource use data were collected in two large, international, multi-centre, randomised controlled trials (RCTs) that studied nintedanib for the treatment of IPF (INPULSIS-1 and -2). The pooled data of nintedanib and placebo included 1014 patients followed for 12 months. The trial data were analysed in 3-month intervals. We studied two dependent variables: the occurrence of allcause hospitalisation and visits to a physician (GP or specialist). The independent variables included the change in forced vital capacity percent predicted (FVC%pred), investigator-reported acute exacerbation events, age, time since diagnosis, smoking status, and sex. Results Hospitalisation during a 3-month interval was significantly associated with a drop of at least 5 or 10 points in FVC%pred (odds ratios [ORs] 1.58 [p = 0.009] and 2.62[p \ 0.001]) and associated with the occurrence of at least one acute exacerbation (OR 14.44; p \ 0.001) during the same interval. The above factors remained significant when repeating the analysis for hospitalisation based on change in FVC%pred or events occurring during the previous 3 months interval. Smoker status and a unit change in FVC%pred during the previous interval were added to the significant factors. Physician visits during a 3-month interval were significantly associated with a lower FVC%pred at the start of the interval (per 10-point decrement, OR 1.05; p = 0.040) and with the change in FVC%pred during the same interval (per 10-point loss, OR 1.13; p = 0.042). Visits were also associated with a 5-point drop in FVC%pred (OR 1.23; p = 0.020), age (per 5-year increments OR 1.07; p = 0.028), and female sex (OR 1.32; p = 0.017). Nevertheless, the predictive power of the models was considered poor for both outcomes (hospitalisation and physician visits). Conclusions Disease progression and acute exacerbation events are significantly associated with hospitalisation of patients with IPF. Outpatient visits to physicians are associated with disease progression, baseline FVC%pred, age and sex.

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Economic Burden of Idiopathic Pulmonary Fibrosis in Spain: A Prospective Real-World Data Study (OASIS Study)

et al. 2023

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Background Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal lung disease associated with dyspnoea, cough and impaired quality of life affecting around 7500 patients in Spain. Objective Our aim was to estimate the economic impact of IPF according to forced vital capacity (FVC) % predicted level in adult patients. Methods We conducted a prospective, observational, multicentric study of patients with confirmed IPF in Spain. Total annual IPF-related costs were estimated per patient, and categorised according to the FVC% predicted value (FVC < 50%, FVC 50–80%, FVC > 80%) and total sample. Incurred direct health- and non-health-related costs and indirect costs were calculated considering the IPF-related healthcare resource use and the corresponding unitarian costs. Results were updated to 2023 euros. Results Two hundred and four consecutive patients with IPF were included: 77% male, average age (standard deviation) 70.8 (7.6) years. At baseline, FVC% was < 50%, 50–80% and > 80% of predicted value in 10.8%, 74.5% and 14.7% of patients, respectively. The final cost-evaluable population included 180 subjects. The mean (standard deviation) total annual IPF-related cost was €26,997 (17,555), with statistically significant differences ( p = 0.0002) between groups: €44,412 (33,389) for the FVC < 50%, €25,803 (14,688) for the FVC 50–80% and €23,242 (13,642) for the FVC > 80%. Annual direct health costs had the greatest weight and included pharmacological treatments [€22,324 (13,773)] and hospitalisation days [€1659 (7362)]. 14 patients had ≥ 1 acute exacerbation of IPF during the study; mean total cost of an acute exacerbation of IPF was €10,372. According to the multivariate analysis, an impaired lung function (FVC < 50%) and use of antifibrotic treatment were determinants of cost ( p < 0.0001 both). Conclusions We observed a significantly higher annual IPF-related cost at a lower level of predicted FVC%, the direct cost having the greatest weight to the total costs. Maintaining patients at early disease stages by slowing IPF progression is relevant to reduce the economic impact of IPF. Clinical Trial Registration EU PAS register number EUPAS19387 (1 June, 2017). Supplementary Information The online version contains supplementary material available at 10.1007/s40273-023-01278-3.

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Guidelines for the Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis

Cited by 17 publications

References 76 publications

Pirfenidone for the treatment of idiopathic pulmonary fibrosis

Pirfenidone for the treatment of idiopathic pulmonary fibrosis

Influence of Idiopathic Pulmonary Fibrosis Progression on Healthcare Resource Use

Economic Burden of Idiopathic Pulmonary Fibrosis in Spain: A Prospective Real-World Data Study (OASIS Study)

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