Body composition, muscle function, and physical performance in fibrotic interstitial lung disease: a prospective cohort study

Guler, Sabina; Hur, Seo Am; Lear, Scott A.; Camp, Pat G.; Ryerson, Christopher J.

doi:10.1186/s12931-019-1019-9

Cited by 42 publications

(45 citation statements)

References 47 publications

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“…Individuals with a more impaired pulmonary function were found to have lower muscle mass and higher fat mass. Furthermore, men but not women in the study were found to have lower handgrip strength than expected compared to an age-matched healthy Canadian population [49].…”

Section: Discussioncontrasting

confidence: 60%

Nutritional status and quality of life in interstitial lung disease: a prospective cohort study

et al. 2021

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Background Malnutrition and altered body composition are well-documented in chronic pulmonary diseases; however, investigation of nutritional status in interstitial lung disease (ILD) is limited. This study aimed to describe the nutritional status of ILD patients within three diagnostic groups and explore the relationship between nutritional status and quality of life (QoL). Methods Consecutive patients attending an ILD clinic within a tertiary referral hospital in Sydney, Australia were studied. Weight, body-mass-index, anthropometrics, handgrip strength (HGS), subjective global assessment and QoL questionnaires (EQ-5D-5L and King’s-Brief Interstitial-Lung-Disease ‘K-BILD’) were collected. Associations between nutritional status and QoL were analysed. Results Ninety participants were recruited and categorised: (1) Idiopathic Pulmonary Fibrosis (IPF) (2) Connective-Tissue Disease associated-ILD (CTD-ILD) or (3) Other (non-IPF/non-CTD ILD). Median age was 66.5 (18) years. Four-percent of patients were underweight and 50% were overweight or obese. Median HGS was 71%-(25.3) of predicted and was correlated to all measures of QoL including EQ-5D health-state index (r = 0.376, p < 0.0001), patient-reported EQ-5D-5L Visual Analogue Score (r = 0.367, p < 0.0001) and K-BILD total score (r = 0.346, p = 0.001). Twenty-three percent of the variance in K-BILD total score (F = 12.888, p < 0.0001) was explained by HGS (ß = 0.273, p = 0.006) and forced vital capacity % predicted (ß = 0.331, p = 0.001). Conclusions Although a small number of ILD patients were malnourished, a large proportion of the cohort were overweight or obese. Handgrip strength was compromised and correlated to QoL. Future research with a larger cohort is required to explore the role of HGS as a predictor of QoL.

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Section: Discussioncontrasting

confidence: 60%

Nutritional status and quality of life in interstitial lung disease: a prospective cohort study

et al. 2021

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“…Pneumococcal vaccination and annual influenza vaccination are also appropriate in almost all patients with fibrotic ILD. With frequent loss of physical function and muscle mass in patients with severe and progressive ILD [38], pulmonary rehabilitation potentially relieves symptoms and improves exercise capacity [39]. Similarly, selected patients can benefit from long-term oxygen therapy [40].…”

Section: Non-pharmacologic Treatmentmentioning

confidence: 99%

Progression of fibrosing interstitial lung disease

2020

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Fibrotic interstitial lung diseases (ILDs) are often challenging to diagnose and classify, but an accurate diagnosis has significant implications for both treatment and prognosis. A subset of patients with fibrotic ILD experience progressive deterioration in lung function, physical performance, and quality of life. Several risk factors for ILD progression have been reported, such as male sex, older age, lower baseline pulmonary function, and a radiological or pathological pattern of usual interstitial pneumonia. Morphological similarities, common underlying pathobiologic mechanisms, and the consistently progressive worsening of these patients support the concept of a progressive fibrosing (PF)-ILD phenotype that can be applied to a variety of ILD subtypes. The conventional approach has been to use antifibrotic medications in patients with idiopathic pulmonary fibrosis (IPF) and immunosuppressive medications in patients with other fibrotic ILD subtypes; however, recent clinical trials have suggested a favourable treatment response to antifibrotic therapy in a wider variety of fibrotic ILDs. This review summarizes the literature on the evaluation and management of patients with PF-ILD, and discusses questions relevant to applying recent clinicial trial findings to real-world practice.

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“…Especially, we found significant genetic correlations between IPF and many body fat related traits. Different studies have found that obesity is a common comorbidity of IPF [38,39]. Lower body mass index (BMI) and body weight loss seemed to be related to poor outcomes [40].…”

Section: Discussionmentioning

confidence: 99%

Integrative Analyses Reveal Novel Disease-associated Loci and Genes for Idiopathic Pulmonary Fibrosis

Chen

Zhang

Adams

et al. 2021

Preprint

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Background: Although genome-wide association studies have identified many genomic regions associated with idiopathic pulmonary fibrosis (IPF), the causal genes and functions remain largely unknown. Many bulk and single-cell expression data have become available for IPF, and there is increasing evidence suggesting a shared genetic basis between IPF and other diseases. Methods: By leveraging shared genetic information and transcriptome data, we conducted an integrative analysis to identify novel genes for IPF. We first considered observed phenotypes, polygenic risk scores, and genetic correlations to investigate associations between IPF and other traits in the UK Biobank. We then performed local genetic correlation analysis and cross-tissue transcriptome-wide association analysis (TWAS) to identify IPF genes. We further prioritized genes using bulk and single-cell gene expression data. Findings: We identified 25 traits correlated with IPF on the phenotype level and seven traits genetically correlated with IPF. Using local genetic correlation, we identified 12 candidate genes across 14 genomic regions, including the POT1 locus (p-value = 4.1E-4), which contained variants with protective effects on lung cancer but increasing IPF risk. Using TWAS, we identified 36 genes, including 12 novel genes for IPF. Annotation-stratified heritability estimation and differential expression analysis of downstream-regulated genes suggested regulatory roles of two candidate genes, MAFK and SMAD2, on IPF. Interpretation: Our integrative analysis identified new genes for IPF susceptibility and expanded the understanding of the complex genetic architecture of IPF. Funding: NIHR Leicester Biomedical Research Centre, Three Lakes Partners, the National Institutes of Health, the National Science Foundation, U01HL145567, and UH2HL123886.

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Body composition, muscle function, and physical performance in fibrotic interstitial lung disease: a prospective cohort study

Cited by 42 publications

References 47 publications

Nutritional status and quality of life in interstitial lung disease: a prospective cohort study

Nutritional status and quality of life in interstitial lung disease: a prospective cohort study

Progression of fibrosing interstitial lung disease

Integrative Analyses Reveal Novel Disease-associated Loci and Genes for Idiopathic Pulmonary Fibrosis

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