Progressive fibrosing interstitial lung disease: treatable traits and therapeutic strategies

Wongkarnjana, Amornpun; Scallan, Ciaran; Kolb, Martin

doi:10.1097/mcp.0000000000000712

Cited by 19 publications

(22 citation statements)

References 43 publications

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“…The small numbers of patients with a certain non-IPF ILD subtype can also be considered a limitation of our study. However, combining different progressive ILD subtypes together to a group called "progressive fibrosing interstitial lung diseases" (PF-ILD) has recently become more common [36][37][38][39][40] and we believe that our study may provide relevant novel important information on the disease course of PF-ILDs.…”

Section: Plos Onementioning

confidence: 99%

Causes of acute respiratory hospitalizations predict survival in fibrosing interstitial lung diseases

Salonen¹,

Vähänikkilä²,

Purokivi³

et al. 2020

PLoS ONE

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Acute exacerbation of ILD (AE-ILD) is a common reason for hospitalization; it is also associated with significant mortality. Less is known about the prognostic significance of other events causing acute, non-elective hospitalizations in ILD patients. ILD patients hospitalized due to acute respiratory worsening were collected from medical records. Reasons for respiratory deterioration were classified into AE-ILDs and other causes. Clinical features and survival data of idiopathic pulmonary fibrosis (IPF) and other types of ILDs were evaluated and compared. In all, 237 patients (138 with IPF and 99 with other ILD) fulfilled the inclusion criteria. Of the non-IPF ILD types, the most prevalent subgroups were connective tissue disease-associated ILD (n = 33) and asbestosis (n = 22). The most common cause for hospitalization was AE-ILD explaining 41% of hospitalizations. Lower respiratory tract infection (22%), subacute progression of ILD (12%) and cardiovascular causes (7.2%) were other common reasons for hospital treatment. Patients with a lower respiratory tract infection had a more favorable prognosis compared with patients with AE-ILD. AE-ILDs were less fatal than cardiovascular or concurrent non-ILD-related causes for hospitalizations in non-IPF patients. High Gender-Age-Physiology (GAP) index was a marker for shortened survival and earlier AE-ILDs in all patients. IPF patients had a significantly shorter overall and post-hospitalization survival time compared with other ILDs. Most respiratory hospitalizations in ILD patients were related to causes other than AE-ILD, which highlights the importance of accurate differential diagnosis in order to target the appropriate treatment for each ILD patient.

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Section: Plos Onementioning

confidence: 99%

Causes of acute respiratory hospitalizations predict survival in fibrosing interstitial lung diseases

Salonen¹,

Vähänikkilä²,

Purokivi³

et al. 2020

PLoS ONE

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“… 39 IPF and PF-ILD have been suggested to share common fibrotic cascades that cause irreversible damage and poor outcomes. 40 During the past few years, the SENSCIS trial has shown that nintedanib reduces the rate of FVC decline in patients with systemic sclerosis-associated ILD. 41 Additionally, the INBUILD trial showed that nintedanib improved the FVC decline in patients with PF-ILD.…”

Section: Discussionmentioning

confidence: 99%

Real-world impact of antifibrotics on prognosis in patients with progressive fibrosing interstitial lung disease

et al. 2023

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ObjectiveNo studies have demonstrated the real-world efficacy of antifibrotics for progressive fibrosing interstitial lung disease (PF-ILD). Therefore, we evaluated the efficacy of antifibrotics in patients with PF-ILD.MethodsWe retrospectively reviewed the medical records of patients with ILD from January 2012 to July 2021. Patients were diagnosed with PF-ILD if they had ≥10% fibrosis on high-resolution CT (HRCT) and a relative forced vital capacity (FVC) decline of either ≥10% or >5% to <10% with clinical deterioration or progression of fibrosis on HRCT during overlapping windows of 2 years and with a %FVC of ≥45%. We compared FVC changes and overall survival (OS) between patients with and without antifibrotics. FVC changes were analysed using generalised estimating equations. We used inverse probability weighting (IPW) and statistical matching to adjust for covariates.ResultsOf the 574 patients, 167 were diagnosed with PF-ILD (idiopathic pulmonary fibrosis (IPF), n=64; non-IPF, n=103). Antifibrotics improved the FVC decline in both IPF (p=0.002) and non-IPF (p=0.05) (IPW: IPF, p=0.015; non-IPF, p=0.031). Among patients with IPF, OS was longer in the antifibrotic group (log-rank p=0.001). However, among patients with non-IPF, OS was not longer in the antifibrotic group (p=0.3263) (IPW and statistical matching: IPF, p=0.0534 and p=0.0018; non-IPF, p=0.5663 and p=0.5618).ConclusionThis is the first real-world study to show that antifibrotics improve the FVC decline in PF-ILD. However, among patients with non-IPF, we found no significant difference in mortality between those with and without antifibrotics. Future studies must clarify whether antifibrotics improve the prognosis of non-IPF.

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“…Hypoxemia, low DLCO% predicted, and HRCT-documented UIP pattern were associated with worse mortality in patients with PF. Reportedly, male sex, older age, lower FVC or DLCO at baseline, decline in lung function compared with the baseline, subgroups based on diagnosis, HRCT-documented UIP-like fibrotic pattern, or honeycombing were indicators of poor prognosis (Wongkarnjana et al, 2020). Cluster analysis was used to classify patients with ILD into clinical subgroups based on their clinical courses.…”

Section: Discussionmentioning

confidence: 99%

“…Several clinical trials have encompassed patients with progressive fibrosing, with the eligibility criteria for these studies helping to guide a standardized diagnosis of PF-ILD (Wongkarnjana et al, 2020). Although no uniform criteria were established, guidelines recommended monitoring of fibrosis progression based on evaluation of multiple components, such as a decline in lung function, increase in chest imaging-documented fibrosis, symptomatic worsening, and composite measures of these variables (George et al, 2020;Wongkarnjana et al, 2020;Raghu et al, 2022).…”

Section: Introductionmentioning

confidence: 99%

Prognostic Predictive Characteristics in Patients With Fibrosing Interstitial Lung Disease: A Retrospective Cohort Study

Wang

Guo

et al. 2022

Front. Pharmacol.

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Background: Limited data are available regarding the entire spectrum of interstitial lung disease with a progressive fibrosing feature. We investigated the prevalence and prognostic predictive characteristics in patients with PF-ILD.Methods: This retrospective cohort study included patients with fibrosing ILD who were investigated between 1 January 2015 and 30 April 2021. We recorded clinical features and outcomes to identify the possible risk factors for fibrosing progression as well as mortality.Results: Of the 579 patients with fibrosing ILD, 227 (39.21%) met the criteria for progression. Clubbing of fingers [odds ratio (OR) 1.52, 95% confidence interval (CI) 1.03 to 2.24, p = 0.035] and a high-resolution computed tomography (HRCT)-documented usual interstitial pneumonia (UIP)-like fibrotic pattern (OR 1.95, 95% CI 1.33 to 2.86, p = 0.001) were risk factors for fibrosis progression. The mortality was worse in patients with PF with hypoxemia [hazard ratio (HR) 2.08, 95% CI 1.31 to 3.32, p = 0.002], in those with baseline diffusion capacity of the lung for carbon monoxide (DLCO) % predicted <50% (HR 2.25, 95% CI 1.45 to 3.50, p < 0.001), or in those with UIP-like fibrotic pattern (HR 1.68, 95% CI 1.04 to 2.71, p < 0.001).Conclusion: Clubbing of fingers and an HRCT-documented UIP-like fibrotic pattern were more likely to be associated with progressive fibrosing with varied prevalence based on the specific diagnosis. Among patients with progressive fibrosing, those with hypoxemia, lower baseline DLCO% predicted, or UIP-like fibrotic pattern showed poor mortality.

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Progressive fibrosing interstitial lung disease: treatable traits and therapeutic strategies

Cited by 19 publications

References 43 publications

Causes of acute respiratory hospitalizations predict survival in fibrosing interstitial lung diseases

Causes of acute respiratory hospitalizations predict survival in fibrosing interstitial lung diseases

Real-world impact of antifibrotics on prognosis in patients with progressive fibrosing interstitial lung disease

Prognostic Predictive Characteristics in Patients With Fibrosing Interstitial Lung Disease: A Retrospective Cohort Study

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