Improved Survival of IPF patients Treated With Antifibrotic Drugs Compared With Untreated Patients

Platenburg, Mark G J P; Moorsel, Coline H.M. van; Wiertz, Ivo A.; Vis, Joanne J. van der; Vorselaars, Adriane D.M.; Veltkamp, Marcel; Grutters, Jan C.

doi:10.1007/s00408-023-00628-4

Cited by 5 publications

(6 citation statements)

References 45 publications

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“…We would like to thank Fujirebio Europe N.V. for providing KL-6 reactives free of charge and David Bridgewater for the English language revision of the manuscript. 5 Respiratory Department, Hospital Moises Broggi. -Sant Joan Despí, Barcelona, Spain…”

Section: Acknowledgementsmentioning

confidence: 99%

“…Idiopathic pulmonary fibrosis (IPF) is a fatal progressive fibrotic interstitial lung disease (ILD) [1][2][3]. Antifibrotic drugs slow the decline of lung function [4] and improve prognosis, which remains poor [5]. Sleep-disordered breathing (SDB) is a highly prevalent IPF comorbidity, ranging from 45 to 90% depending on the methodology used [6][7][8][9][10][11].…”

Section: Introductionmentioning

confidence: 99%

“…The treatment of SDB in IPF patients seems to improve quality of life and prognosis despite the scarce number of studies [11,22,23]. Some methodological limitations include the absence of a generalized use of antifibrotic drug therapy [4,5] and self-selection bias owing to a high refusal rate and poor compliance with continuous positive airway pressure (CPAP) [24]. There is limited data regarding the effect of using nocturnal oxygen therapy (NOT) in ILD patients and, to the best of our knowledge, there is no data regarding the effect of treating CSA in patients with ILD [25][26][27].…”

Section: Introductionmentioning

confidence: 99%

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Treating sleep-disordered breathing of idiopathic pulmonary fibrosis patients with CPAP and nocturnal oxygen treatment. A pilot study

Bordas-Martinez,

Salord,

Vicens-Zygmunt

et al. 2024

Respir Res

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Introduction Sleep-disordered breathing (SDB) is a major comorbidity in idiopathic pulmonary fibrosis (IPF) and is associated with a poor outcome. There is a lack of knowledge regarding the impact of SDB treatment on IPF. We assessed at one year: (1) the effect of CPAP and/or nocturnal oxygen therapy on IPF regarding lung function, blood mediators, and quality of life; (2) adherence to SDB treatment and SDB changes. Methodology This is a prospective study of consecutive newly diagnosed IPF patients initiating anti-fibrotic treatment. Lung function, polysomnography, blood tests and quality of life questionnaires were performed at inclusion and after one year. Patients were classified as obstructive sleep apnoea (OSA), central sleep apnoea (CSA), and sleep-sustained hypoxemia (SSH). SDB therapy (CPAP and/or nocturnal oxygen therapy) was initiated if needed. Results Fifty patients were enrolled (36% had OSA, 22% CSA, and 12% SSH). CPAP was started in 54% of patients and nocturnal oxygen therapy in 16%. At one-year, polysomnography found improved parameters, though 17% of patients had to add nocturnal oxygen therapy or CPAP, while 33% presented SDB onset at this second polysomnography. CPAP compliance at one year was 6.74 h/night (SD 0.74). After one year, matrix metalloproteinase-1 decreased in OSA and CSA (p = 0.029; p = 0.027), C-reactive protein in OSA (p = 0.045), and surfactant protein D in CSA group (p = 0.074). There was no significant change in lung function. Conclusions Treatment of SBD with CPAP and NOT can be well tolerated with a high compliance. IPF patients may exhibit SDB progression and require periodic re-assessment. Further studies to evaluate the impact of SDB treatment on lung function and serological mediators are needed.

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Section: Acknowledgementsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Treating sleep-disordered breathing of idiopathic pulmonary fibrosis patients with CPAP and nocturnal oxygen treatment. A pilot study

Bordas-Martinez,

Salord,

Vicens-Zygmunt

et al. 2024

Respir Res

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“…Real-world data, although inconsistent, have demonstrated that approved IPF therapies ( i.e. pirfenidone and nintedanib) are associated with improvements in survival [ 46 – 49 ]. For example, one study of 457 patients reported transplant-free survival rates of 3.4 versus 2.2 years with approved treatment versus without, respectively (p=0.005) [ 46 ].…”

Section: Role Of Atx and Lpar1 Signalling In Fibrotic Diseasesmentioning

confidence: 99%

Lysophosphatidic acid receptor 1 inhibition: a potential treatment target for pulmonary fibrosis

Volkmann,

Denton,

Kolb

et al. 2024

Eur Respir Rev

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Lysophosphatidic acid (LPA)-mediated activation of LPA receptor 1 (LPAR1) contributes to the pathophysiology of fibrotic diseases such as idiopathic pulmonary fibrosis (IPF) and systemic sclerosis (SSc). These diseases are associated with high morbidity and mortality despite current treatment options. The LPA-producing enzyme autotaxin (ATX) and LPAR1 activation contribute to inflammation and mechanisms underlying fibrosis in preclinical fibrotic models. Additionally, elevated levels of LPA have been detected in bronchoalveolar lavage fluid from patients with IPF and in serum from patients with SSc. Thus, ATX and LPAR1 have gained considerable interest as pharmaceutical targets to combat fibrotic disease and inhibitors of these targets have been investigated in clinical trials for IPF and SSc. The goals of this review are to summarise the current literature on ATX and LPAR1 signalling in pulmonary fibrosis and to help differentiate the novel inhibitors in development. The mechanisms of action of ATX and LPAR1 inhibitors are described and preclinical studies and clinical trials of these agents are outlined. Because of their contribution to numerous physiologic events underlying fibrotic disease, ATX and LPAR1 inhibition presents a promising therapeutic strategy for IPF, SSc and other fibrotic diseases that may fulfil unmet needs of the current standard of care.

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“…Значительно более низкая куму лятивная смертность от ИЛФ в течение 1, 2 и 3 лет обнаружена при назначении АФТ (1 год -7,0 % vs 35,6 %; 2 года -26,6 % vs 55,9 %; 3 года -46,9 % vs 55,9 %). По результатам этого масштабного исследо вания, проведенного в условиях реальной практики, показано улучшение выживаемости при назначении АФТ больным ИЛФ по сравнению с таковой у паци ентов, не получающих АФТ [19].…”

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The effectiveness of early administration of antifibrotic therapy as a reflection of properly organized medical care for patients with progressive pulmonary fibrosis

Ignatova,

Antonov,

Blinova

et al. 2024

Pulʹmonologiâ (Mosk.)

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The issues of organization of medical care and early administration of antifibrotic therapy in patients with progressive fibrotic phenotype require in-depth study and implementation in real clinical practice.The aim of this paper is to provide a rationale for the early administration of antifibrotic drugs in patients with various forms of interstitial lung disease.Results. The article addresses organizational issues in the medical care for interstitial lung disease with a progressive fibrotic phenotype. Topical epidemiological problems are also addressed, in particular the high mortality rate in this disease. The main risk factors of the disease are considered. Diagnostic markers for the fibrosis progression are analyzed. Data from large-scale studies on the early initiation of antifibrotic therapy and its effect on disease outcomes are presented.Conclusion. The main reasons for early initiation of therapy in a progressive fibrotic phenotype are presented: an unfavorable prognosis, an unpredictable course, a progressive decrease in forced vital capacity, and the association of changes in FVC with an increase in mortality. Using the Chelyabinsk region as an example, a model of care for patients with interstitial lung diseases is considered, with a brief analysis of the effectiveness of antifibrotic therapy, its early initiation and the impact on mortality in patients with this disease.

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Improved Survival of IPF patients Treated With Antifibrotic Drugs Compared With Untreated Patients

Cited by 5 publications

References 45 publications

Treating sleep-disordered breathing of idiopathic pulmonary fibrosis patients with CPAP and nocturnal oxygen treatment. A pilot study

Treating sleep-disordered breathing of idiopathic pulmonary fibrosis patients with CPAP and nocturnal oxygen treatment. A pilot study

Lysophosphatidic acid receptor 1 inhibition: a potential treatment target for pulmonary fibrosis

The effectiveness of early administration of antifibrotic therapy as a reflection of properly organized medical care for patients with progressive pulmonary fibrosis

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