Association of musculoskeletal involvement with lung function and mortality in patients with idiopathic pulmonary fibrosis

Abstract Idiopathic pulmonary fibrosis (IPF) is a progressive disease associated with high mortality. Low muscle mass, frailty and sarcopenia lead to functional impairment that negatively impact quality of life and survival but are not used in clinical practice. We aimed to determine the association...

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Main Authors: Meenakshi Sridhar, Sandeep Bodduluri, Lanier O’Hare, Scott Blumhoff, Maria del Pilar Acosta Lara, Joao A. de Andrade, Young-Il Kim, Tracy Luckhardt, MerryLynn McDonald, Tejaswini Kulkarni
Format: Article
Language:English
Published: BMC 2024-02-01
Series:Respiratory Research
Subjects:
Online Access:https://doi.org/10.1186/s12931-024-02705-5
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author Meenakshi Sridhar
Sandeep Bodduluri
Lanier O’Hare
Scott Blumhoff
Maria del Pilar Acosta Lara
Joao A. de Andrade
Young-Il Kim
Tracy Luckhardt
MerryLynn McDonald
Tejaswini Kulkarni
author_facet Meenakshi Sridhar
Sandeep Bodduluri
Lanier O’Hare
Scott Blumhoff
Maria del Pilar Acosta Lara
Joao A. de Andrade
Young-Il Kim
Tracy Luckhardt
MerryLynn McDonald
Tejaswini Kulkarni
author_sort Meenakshi Sridhar
collection DOAJ
description Abstract Idiopathic pulmonary fibrosis (IPF) is a progressive disease associated with high mortality. Low muscle mass, frailty and sarcopenia lead to functional impairment that negatively impact quality of life and survival but are not used in clinical practice. We aimed to determine the association of Fat-free mass index (FFMI) and frailty with lung function, exercise tolerance and survival in patients with IPF. In this study, 70 patients with IPF underwent assessment of body composition, lung function, 6-min walk distance (6MWD) testing, hand grip strength, quality of life (QoL) assessment by St. George’s Respiratory questionnaire (SGRQ) and frailty assessment using the SHARE-FI tool. FFMI was calculated using pectoralis muscle cross-sectional area (PM-CSA) on CT chest images and the lowest quartile defined reduced muscle mass. Sarcopenia was defined as low FFMI and handgrip strength. Regression analyses were conducted to determine predictive value of frailty, low FFMI and sarcopenia on clinical outcomes. The Cox proportional hazards model was used to analyze the impact of FFMI and frailty score on survival. The mean age was 70 years with moderate impairment in lung function (mean ppFVC 68.5%, ppDLCO 45.6%). Baseline forced vital capacity (p < 0.001), diffusion capacity of lung for carbon monoxide (p =  < 0.01), 6WMD (p < 0.05) were significantly lower in frail patients compared to non-frail patients. BMI was found to closely correlate with FFMI (r = 0.79, p < 0.001), but not with frailty score (r = − 0.2, p = 0.07). Frailty was a significant predictor of FVC, DLCO, 6MWD, SGRQ scores when adjusted for age and gender. Muscle mass and sarcopenia were significant predictors of FVC, DLCO, but not 6MWD or QoL scores. Multivariate cox-proportional hazards ratio model adjusting for age and gender showed that frailty was significantly associated with increased mortality (HR = 2.6, 95% CI 1.1–6.1). Low FFMI (HR = 1.3, 95% CI 0.6–2.8), and sarcopenia (HR = 2.1, 95% CI 0.8–5.3), though associated with a trend to increased mortality, were not statistically significant. Frailty is associated with lower lung function and higher mortality in patients with IPF. Longitudinal evaluations are necessary to further determine the associations between low FFMI, sarcopenia and frailty with outcomes in IPF.
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spelling doaj.art-b00b000a77264f42b3b46003b5f1812e2024-03-05T20:02:26ZengBMCRespiratory Research1465-993X2024-02-012511910.1186/s12931-024-02705-5Association of musculoskeletal involvement with lung function and mortality in patients with idiopathic pulmonary fibrosisMeenakshi Sridhar0Sandeep Bodduluri1Lanier O’Hare2Scott Blumhoff3Maria del Pilar Acosta Lara4Joao A. de Andrade5Young-Il Kim6Tracy Luckhardt7MerryLynn McDonald8Tejaswini Kulkarni9Division of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine, School of Medicine, University of Alabama at BirminghamDivision of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine, School of Medicine, University of Alabama at BirminghamDivision of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine, School of Medicine, University of Alabama at BirminghamLehigh Valley Health NetworkDivision of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine, School of Medicine, University of Alabama at BirminghamDivision of Pulmonary, Allergy and Critical Care Medicine, Vanderbilt UniversityDivision of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine, School of Medicine, University of Alabama at BirminghamDivision of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine, School of Medicine, University of Alabama at BirminghamDivision of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine, School of Medicine, University of Alabama at BirminghamDivision of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine, School of Medicine, University of Alabama at BirminghamAbstract Idiopathic pulmonary fibrosis (IPF) is a progressive disease associated with high mortality. Low muscle mass, frailty and sarcopenia lead to functional impairment that negatively impact quality of life and survival but are not used in clinical practice. We aimed to determine the association of Fat-free mass index (FFMI) and frailty with lung function, exercise tolerance and survival in patients with IPF. In this study, 70 patients with IPF underwent assessment of body composition, lung function, 6-min walk distance (6MWD) testing, hand grip strength, quality of life (QoL) assessment by St. George’s Respiratory questionnaire (SGRQ) and frailty assessment using the SHARE-FI tool. FFMI was calculated using pectoralis muscle cross-sectional area (PM-CSA) on CT chest images and the lowest quartile defined reduced muscle mass. Sarcopenia was defined as low FFMI and handgrip strength. Regression analyses were conducted to determine predictive value of frailty, low FFMI and sarcopenia on clinical outcomes. The Cox proportional hazards model was used to analyze the impact of FFMI and frailty score on survival. The mean age was 70 years with moderate impairment in lung function (mean ppFVC 68.5%, ppDLCO 45.6%). Baseline forced vital capacity (p < 0.001), diffusion capacity of lung for carbon monoxide (p =  < 0.01), 6WMD (p < 0.05) were significantly lower in frail patients compared to non-frail patients. BMI was found to closely correlate with FFMI (r = 0.79, p < 0.001), but not with frailty score (r = − 0.2, p = 0.07). Frailty was a significant predictor of FVC, DLCO, 6MWD, SGRQ scores when adjusted for age and gender. Muscle mass and sarcopenia were significant predictors of FVC, DLCO, but not 6MWD or QoL scores. Multivariate cox-proportional hazards ratio model adjusting for age and gender showed that frailty was significantly associated with increased mortality (HR = 2.6, 95% CI 1.1–6.1). Low FFMI (HR = 1.3, 95% CI 0.6–2.8), and sarcopenia (HR = 2.1, 95% CI 0.8–5.3), though associated with a trend to increased mortality, were not statistically significant. Frailty is associated with lower lung function and higher mortality in patients with IPF. Longitudinal evaluations are necessary to further determine the associations between low FFMI, sarcopenia and frailty with outcomes in IPF.https://doi.org/10.1186/s12931-024-02705-5FrailtySarcopeniaFat-free Mass index (FFMI)Interstitial lung diseasePectoralis Muscle AreaBody mass index
spellingShingle Meenakshi Sridhar
Sandeep Bodduluri
Lanier O’Hare
Scott Blumhoff
Maria del Pilar Acosta Lara
Joao A. de Andrade
Young-Il Kim
Tracy Luckhardt
MerryLynn McDonald
Tejaswini Kulkarni
Association of musculoskeletal involvement with lung function and mortality in patients with idiopathic pulmonary fibrosis
Respiratory Research
Frailty
Sarcopenia
Fat-free Mass index (FFMI)
Interstitial lung disease
Pectoralis Muscle Area
Body mass index
title Association of musculoskeletal involvement with lung function and mortality in patients with idiopathic pulmonary fibrosis
title_full Association of musculoskeletal involvement with lung function and mortality in patients with idiopathic pulmonary fibrosis
title_fullStr Association of musculoskeletal involvement with lung function and mortality in patients with idiopathic pulmonary fibrosis
title_full_unstemmed Association of musculoskeletal involvement with lung function and mortality in patients with idiopathic pulmonary fibrosis
title_short Association of musculoskeletal involvement with lung function and mortality in patients with idiopathic pulmonary fibrosis
title_sort association of musculoskeletal involvement with lung function and mortality in patients with idiopathic pulmonary fibrosis
topic Frailty
Sarcopenia
Fat-free Mass index (FFMI)
Interstitial lung disease
Pectoralis Muscle Area
Body mass index
url https://doi.org/10.1186/s12931-024-02705-5
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