Abstract

BackgroundFibrotic interstitial lung disease (FILD) patients are typically dyspneic and exercise-intolerant with consequent impairment of health-related quality of life (HRQoL). Respiratory muscle dysfunction is among the underlying mechanisms of dyspnea and exercise intolerance in FILD but may be difficult to diagnose. Using ultrasound, we compared diaphragmatic mobility and thickening in FILD cases and healthy controls and correlated these findings with dyspnea, exercise tolerance, HRQoL and lung function.MethodsWe measured diaphragmatic mobility and thickness during quiet (QB) and deep breathing (DB) and calculated thickening fraction (TF) in 30 FILD cases and 30 healthy controls. We correlated FILD cases’ diaphragmatic findings with dyspnea, exercise tolerance (six-minute walk test), lung function and HRQoL (St. George’s Respiratory Questionnaire).ResultsDiaphragmatic mobility was similar between groups during QB but was lower in FILD cases during DB when compared to healthy controls (3.99 cm vs 7.02 cm; p < 0.01). FILD cases showed higher diaphragm thickness during QB but TF was lower in FILD when compared to healthy controls (70% vs 188%, p < 0.01). During DB, diaphragmatic mobility and thickness correlated with lung function, exercise tolerance and HRQoL, but inversely correlated with dyspnea. Most FILD cases (70%) presented reduced TF, and these patients had higher dyspnea and exercise desaturation, lower HRQoL and lung function.ConclusionCompared to healthy controls, FILD cases present with lower diaphragmatic mobility and thickening during DB that correlate to increased dyspnea, decreased exercise tolerance, worse HRQoL and worse lung function. FILD cases with reduced diaphragmatic thickening are more dyspneic and exercise-intolerant, have lower HRQoL and lung function.

Highlights

  • Fibrotic interstitial lung disease (FILD) patients are typically dyspneic and exercise-intolerant with consequent impairment of health-related quality of life (HRQoL)

  • The diaphragmatic mobility and thickness were measured for all subjects

  • Respiratory muscle strength was similar between fibrotic interstitial lung disease (FILD) cases and healthy controls (Table 1)

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Summary

Introduction

Fibrotic interstitial lung disease (FILD) patients are typically dyspneic and exercise-intolerant with consequent impairment of health-related quality of life (HRQoL). Respiratory muscle dysfunction is among the underlying mechanisms of dyspnea and exercise intolerance in FILD but may be difficult to diagnose. Dyspnea and exercise intolerance characterize fibrotic interstitial lung diseases (FILD) [1, 2]. Both are determinants of impaired health-related quality of life (HRQoL) observed in FILD patients [1, 3, 4]. Among them is respiratory muscle dysfunction [6, 7], which is usually difficult to diagnose [6]. While some authors have found preserved inspiratory muscle strength in ILD [5, 8,9,10], Walterspachen et al [7] found reduced diaphragmatic strength, assessed with non-volitional measures, in a subset of ILD subjects with severe disease

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