Treatment of progressive fibrosing interstitial lung diseases: a milestone in the management of interstitial lung diseases

Cottin, Vincent

doi:10.1183/16000617.0109-2019

Cited by 56 publications

(46 citation statements)

References 39 publications

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“…The definitions of disease progression of ILD vary across studies. [ 13 ] In most clinical trials or observational studies, the rate of decline in forced vital capacity (FVC) was used to assess disease progression in ILD patients. [ 14 – 16 ] Furthermore, gas exchange parameters, including the diffusing capacity of the lung for carbon monoxide (DLco), worsening of symptoms, exercise capacity, deterioration in health-related quality of life, the extent of lung fibrosis on high-resolution computed tomography (HRCT), or the initiation of supplemental oxygen therapy, were used to assess disease progression.…”

Section: Introductionmentioning

confidence: 99%

Clinical characteristics of non-idiopathic pulmonary fibrosis, progressive fibrosing interstitial lung diseases

Komatsu¹,

Yamamoto²,

Kitaguchi³

et al. 2021

Medicine

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Progressive fibrosing interstitial lung disease (PF-ILD) is a progressive phenotype of fibrosing ILDs with varying definitions and elusive clinical characteristics. We aimed to clarify the clinical features and prognosis of PF-ILD cases based on the deterioration of pulmonary function. Altogether, 91 consecutive ILD patients who underwent at least 2 pulmonary function tests (PFTs) with an interval of at least 24 months, as the screening period, between January 2009 and December 2015 were retrospectively reviewed. The deterioration of forced vital capacity (FVC) and diffusing capacity of the lung for carbon monoxide (DLco) was calculated based on PFT data and screening period. The definition of PF-ILD was relative decline of 10% or more in FVC per 24 months or relative decline in FVC of 5% or more with decline in DLco of 15% or more per 24 months. Medical records of 34 patients with idiopathic pulmonary fibrosis (IPF), 11 patients with non-IPF, PF-ILD, and 46 patients with non-IPF, non-PF-ILD were retrospectively analyzed. Patient characteristics, pharmacologic or non-pharmacologic treatment status, and prognosis were compared between the IPF and non-IPF groups and between the non-IPF, PF-ILD and non-IPF, non-PF-ILD groups. Eleven patients (19.3%) showed a progressive phenotype in the non-IPF group. The pulmonary function data at the first PFT were worse in non-IPF, PF-ILD patients than in non-IPF, non-PF-ILD patients. There were no differences in the proportion of patients who were observed without pharmacologic treatment or of those receiving pharmacologic treatment between the non-IPF, PF-ILD and non-IPF, non-PF-ILD groups. Low %FVC at the first PFT and the usual interstitial pneumonia-like fibrotic pattern on high-resolution computed tomography were risk factors for PF-ILD in the non-IPF group. The mortality in the non-IPF, PF-ILD group was significantly worse than that of the non-IPF, non-PF-ILD group and was as poor as that of the IPF group. Multivariate logistic analysis showed that aging and low %DLco at the first PFT were risk factors for mortality within the non-IPF group. The prognosis of non-IPF, PF-ILD patients was as poor as that of IPF patients. Non-IPF, PF-ILD patients require more intensive treatment before disease progression.

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Section: Introductionmentioning

confidence: 99%

Clinical characteristics of non-idiopathic pulmonary fibrosis, progressive fibrosing interstitial lung diseases

Komatsu¹,

Yamamoto²,

Kitaguchi³

et al. 2021

Medicine

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“…This phenotypically related group of conditions, where progression of disease is similar to that seen in IPF, have recently been described as Progressive Fibrotic Interstitial Lung diseases (PF-ILD) [2]. Historically treatments for these cases have been limited and clinicians, recognising the related progression between these conditions and IPF may have been pragmatically relabelling these cases as IPF based on their disease behaviour to qualify for anti-fibrotic therapy [3]. The INBUILD trial broadened the scope of treatable fILD by demonstrating a significant benefit of Nintedanib in patients with fILD and progressive disease [4].…”

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confidence: 99%

The burden of Progressive Fibrotic Interstitial lung disease across the UK

Simpson

Barratt

Beirne

et al. 2020

Preprint

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While Idiopathic pulmonary fibrosis (IPF) remains the exemplar progressive fibrotic lung disease, there remains a cohort of non-IPF fibrotic lung diseases (fILD) which adopt a similar clinical behaviour to IPF despite therap. This phenotypically related group of conditions, where progression of disease is similar to that seen in IPF, have recently been described as Progressive Fibrotic Interstitial Lung diseases (PF-ILD). Previous estimates suggest that between 18 to 40% of all fILD will develop progressive disease, however, the exact burden remains unknown. This retrospective, observational study therefore aimed to estimate the incidence of PF-ILD across England.All new referrals seen across nine UK centres for their first outpatient clinic appointment between 1st August 2017 and 31st January 2018 were assessed against the diagnostic criteria for PF-ILD laid out in the INBUILD trial. A total of 1749 patients with fILD were assessed. In this cohort of patients at risk of developing PF-ILD the INBUILD criteria were met in 14.5% (253/1749) of all new non-IPF fILD referrals. The average time from referral to specialist centre to diagnosis of progressive phenotype was 311 days. Of the progression events the majority were driven by a measured drop in FVC, with more than half of patients experiencing a drop of 10%. Almost one quarter of patients (24.1%) were diagnosed with progressive disease on the basis of radiological and symptomatic progression alone without a spirometric deterioration.This study represents a fair and balanced approach to assessing the incidence of objectively measurable and treatable PF-ILDs in the UK. A rate of 14.5% of new referrals with non-IPF ILD is less than that reported in previous studies however our methodology is likely to give a more accurate result than estimates based on extrapolation from general disease statistics, from physician-reported estimates prone to significant biases, or insurance claim processes also substantially prone to bias. This information has implication for workforce planning and the funding of anti-fibrotic therapy in the UK and beyond.

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“…Progressive pulmonary fibrosis is the hallmark of IPF, but this phenotype occurs in other ILDs [13,[133][134][135][136].…”

Section: Other Progressive Fibrosing Ildsmentioning

confidence: 99%

Ongoing challenges in pulmonary fibrosis and insights from the nintedanib clinical programme

et al. 2020

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The approvals of nintedanib and pirfenidone changed the treatment paradigm in idiopathic pulmonary fibrosis (IPF), and increased our understanding of the underlying disease mechanisms. Nonetheless, many challenges and unmet needs remain in the management of patients with IPF and other progressive fibrosing interstitial lung diseases. This review describes how the nintedanib clinical programme has helped to address some of these challenges. Data from this programme have informed changes to the IPF diagnostic guidelines, the timing of treatment initiation, and the assessment of disease progression. The use of nintedanib to treat patients with advanced lung function impairment, concomitant emphysema, patients awaiting lung transplantation and patients with IPF and lung cancer is discussed. The long-term use of nintedanib and an up-to-date summary of nintedanib in clinical practice are discussed. Directions for future research, namely emerging therapeutic options, precision medicine and other progressive fibrosing interstitial lung diseases, are described. Further developments in these areas should continue to improve patient outcomes.

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Treatment of progressive fibrosing interstitial lung diseases: a milestone in the management of interstitial lung diseases

Cited by 56 publications

References 39 publications

Clinical characteristics of non-idiopathic pulmonary fibrosis, progressive fibrosing interstitial lung diseases

Clinical characteristics of non-idiopathic pulmonary fibrosis, progressive fibrosing interstitial lung diseases

The burden of Progressive Fibrotic Interstitial lung disease across the UK

Ongoing challenges in pulmonary fibrosis and insights from the nintedanib clinical programme

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