Prognostic factors and disease behaviour of pathologically proven fibrotic non‐specific interstitial pneumonia

Yamakawa, Hideaki; Kitamura, Hideya; Takemura, Tamiko; Ikeda, Satoshi; Sekine, Akimasa; Baba, Tomohisa; Iwasawa, Tae; Hagiwara, Eri; Sato, Shinji; Ogura, Takashi

doi:10.1111/resp.13313

Cited by 22 publications

(18 citation statements)

References 33 publications

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“…The term “PF-ILD” describes chronic fibrosing ILDs and mostly includes IPF, iNSIP, [ 32 ] unclassifiable ILD, [ 9 ] CTD-ILDs, including rheumatoid arthritis-associated ILD, [ 33 ] systemic sclerosis-associated ILD, [ 34 ] chronic hypersensitivity pneumonitis, [ 35 ] and exposure-related disease. [ 12 , 13 ] The definition of PF-ILD varies across studies; therefore, there is no consensus on the definition of disease progression in patients with ILDs.…”

Section: Discussionmentioning

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: Discussionmentioning

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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“…Progression is highly heterogeneous: some cases can remain stable or improve, but others can progress to end-stage fibrosis [16,17,30]. A disease-behaviour classification is therefore useful to manage patients with iNSIP [35]. Serial HRCT images from a patient with NSIP are shown in figure 3.…”

Section: Diagnostic Methodsmentioning

confidence: 99%

Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases

et al. 2018

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@ERSpublicationsOther chronic ILDs with a progressive-fibrosing phenotype may have a clinical course similar to IPF. Although challenging, identification of these patients is crucial, and requires a multidisciplinary approach, to ensure optimal diagnosis and management.ABSTRACT Although these conditions are rare, a proportion of patients with interstitial lung diseases (ILDs) may develop a progressive-fibrosing phenotype. Progressive fibrosis is associated with worsening respiratory symptoms, lung function decline, limited response to immunomodulatory therapies, decreased quality of life and, potentially, early death. Idiopathic pulmonary fibrosis may be regarded as a model for other progressive-fibrosing ILDs. Here we focus on other ILDs that may present a progressive-fibrosing phenotype, namely idiopathic nonspecific interstitial pneumonia, unclassifiable idiopathic interstitial pneumonia, connective tissue disease-associated ILDs (e.g. rheumatoid arthritis-related ILD), fibrotic chronic hypersensitivity pneumonitis, fibrotic chronic sarcoidosis and ILDs related to other occupational exposures. Differential diagnosis of these ILDs can be challenging, and requires detailed consideration of clinical, radiological and histopathological features. Accurate and early diagnosis is crucial to ensure that patients are treated optimally.

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“…ILD disease outcome was also measured based on relative changes in functional parameters (FVC and DLco) between Year 0 and Year 5. While greater than 10% decreases in the ratio of Year 5/Year 0 FVC and/or DLco were de ned as worse, greater than 10% increases in the ratio of Year 5/Year 0 FVC and/or DLco were classi ed as improved and less than 10% change in the Year 5/Year 0 FVC and/or DLco were considered unchanged/stable 18,19 .…”

Section: Imaging and Scoringmentioning

confidence: 99%

Predictors of Long-term Prognosis in Rheumatoid Arthritis-related Interstitial Lung Disease

chen

Chen

Liu

et al. 2021

Preprint

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Objective. To identify specific clinical and serum protein biomarkers that are associated with longitudinal outcome of RA-associated interstitial lung disease（RA-ILD).Methods. 60 RA patients with clinical and serological profiles were assessed by HRCT and pulmonary function tests (PFTs) at baseline (Year 0) and 5 years post enrollment (Year 5). Progression versus non-progression was defined based on the changes in Quantitative Modified HRCT scores and PFTs over time. Specific serum protein biomarkers were assessed in serum samples at baseline and Year 5 by Multiplex enzyme-linked immunosorbent assays (ELISAs).Results. Based on changes of Quantitative Modified HRCT scores, 32% of patients demonstrated progressive RA-ILD, 35% were stable, and 33% improved. Baseline age and rheumatoid factor (RF) were significantly different between RA-ILD outcomes of progression versus no-progression (p=0.002 and 0.027, respectively). In multivariate analyses, changes in the serum levels of CXCL11/I-TAC and MMP13 from Year 0 to Year 5 (log Year 5-log Year 0) also distinguished RA-ILD outcomes (p< 0.05). A final binary logistic regression model revealed that baseline age and changes of MMP13 were associated with RA-ILD progression (Yes vs. No) at Year 5 (p< 0.05). Receiver Operating Characteristics analysis indicated that these variables predicted progression with an AUC of 0.7569.Conclusion. While baseline age and RF predicted RA-ILD outcomes of progression versus no-progression, changed levels of CXCL11/I-TAC and MMP-13 over 5 years were also correlated with long-term prognosis of RA-ILD. In multivariate analyses, baseline age and changed levels of MMP-13 were associated with the risk of progression of RA-ILD.Trial registrationNo

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Prognostic factors and disease behaviour of pathologically proven fibrotic non‐specific interstitial pneumonia

Cited by 22 publications

References 33 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

Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases

Predictors of Long-term Prognosis in Rheumatoid Arthritis-related Interstitial Lung Disease

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