Ventilation distribution and small airway function in patients with systemic sclerosis

Silva, B.R.A.; Rufino, Rogério; Costa, Cláudia Henrique da; Vilela, Verônica Silva; Levy, Roger A.; Lopes, Agnaldo José

doi:10.1016/j.rppnen.2017.01.004

Cited by 8 publications

(13 citation statements)

References 27 publications

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“…Similar to the study by Silva et al (10), we observed that an increased phase III slope N2SBW , which is an indicator of inhomogeneity in ventilation distribution, was the most common change in lung function, suggesting the potential of this index as a marker of lung damage in SSc-ILD patients. High phase III slope N2SBW values indicate a poor ventilation distribution, because this variable measures regional differences in respiratory system time constants due to changes in airway resistance and lung distensibility, which in turn compromise alveolar emptying.…”

Section: Discussionsupporting

confidence: 90%

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Ventilation distribution as a contributor to the functional exercise capacity in patients with systemic sclerosis-associated interstitial lung disease without pulmonary hypertension

Andrade

Oliveira

Lopes

2019

Braz J Med Biol Res

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Phenotypic differences have been described between patients with systemic sclerosis (SSc)-associated interstitial lung disease (ILD) and SSc-associated pulmonary hypertension, including performance differences in the 6-min walk test (6MWT). Moreover, the correlations between the 6MWT and traditional pulmonary function tests (PFTs) are weak, indicating the need to search for new parameters that explain exercise performance. Thus, our objective was to evaluate the impact of ventilation distribution heterogeneity assessed by the nitrogen single-breath washout (N 2 SBW) test and peripheral muscle dysfunction on the exercise capacity in patients with SSc-ILD and limited involvement of the pulmonary parenchyma. In this cross-sectional study, 20 women with SSc-ILD and 20 matched controls underwent PFTs (including spirometry, diffusing capacity for carbon monoxide (DLco), and the N 2 SBW test) and performed the 6MWT and knee isometric dynamometry. The 6-min walking distance (6MWD, % predicted) was strongly correlated with the phase III slope of the single-breath nitrogen washout (phase III slope N2SBW ) (r=−0.753, P<0.0001) and reasonably correlated with the forced vital capacity (FVC) (r=0.466, P=0.008) and DLco (r=0.398, P=0.011). The peripheral oxygen saturation (SpO 2 ) during exercise was not significantly correlated with any of the pulmonary or muscle function parameters. The phase III slope N2SBW was the only predictive variable for the 6MWD, whereas quadriceps strength and FVC/DLco were predictive variables for SpO 2 . Ventilation distribution heterogeneity is one factor that contributes to a lower 6MWD in SSc-ILD patients. In addition, muscle dysfunction and abnormal lung diffusion at least partly explain the decreased SpO 2 of these patients.

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Section: Discussionsupporting

confidence: 90%

“…A recent study used the N 2 SBW test in SSc patients. The results showed that heterogeneity in the ventilation distribution was a frequent finding in these patients and that this change was found even in the absence of restrictive damage based on PFTs (10).…”

Section: Introductionmentioning

confidence: 95%

Ventilation distribution as a contributor to the functional exercise capacity in patients with systemic sclerosis-associated interstitial lung disease without pulmonary hypertension

Andrade

Oliveira

Lopes

2019

Braz J Med Biol Res

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“…They found that SIII was the most sensitive pulmonary function alteration in these patients, even when other pulmonary function tests were normal. 29 The SBWN 2 test showed heterogeneity in pulmonary ventilation in all three conditions (IPF, CPFE and COPD), without large differences between groups, which did not aid in the functional differentiation between IPF and CPFE. Additionally, our data suggested that patients with CPFE experienced signi cant air trapping, even when the lung volumes were within normal limits.…”

Section: Discussionmentioning

confidence: 86%

Periostin and closing volume in combined pulmonary fibrosis and emphysema

Anselmo

Costa

Rodrigues

et al. 2020

Preprint

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Background Combined pulmonary fibrosis and emphysema (CPFE) is an entity characterized by the presence of emphysema in upper lobes and fibrosis in lower lobes. Due to the presence of the two diseases concomitantly, it may be difficult to diagnose. This study aims at a better understanding of this entity and proposes biological markers (functional and biochemical) that help in this characterization. Methods A prospective, observational, cross-sectional study was carried out at a reference center. Pulmonary function tests (spirometry, CO-diffusion capacity, plethysmography and single-maneuver nitrogen washout test - SBWN 2 ) and biochemical markers (periostin, mucin-16, PDGF-BB and TGF-β 1 ) were measured in groups of patients: idiopathic pulmonary fibrosis, CPFE and chronic obstructive pulmonary disease (COPD). Results Variables derived from SBWN 2 - closing volume (CV) / vital capacity (VC) (%) and closing capacity (CC) / total lung capacity (TLC) (%) - were found to be higher in the CPFE group compared to the Idiopathic pulmonary fibrosis (IPF) group (CV/VC%: 0.25 (0.12 – 11.01) and 13.05 (0.21 – 20.73); p = 0.005; CC/TLC%: 30.1 (22.4 – 37.47) and 33.69 (32.05 – 41.98); p = 0.03, respectively). Periostin was higher in the CPFE group than in the other groups [CPFE: 66.74 (45.21 – 90.5), IPF: 43.81 (31.97 – 56.18), COPD: 40.08 (20.66 – 50.81); p = 0.0002], and mucin-16 was higher in the IPF group than in the CPFE group [CPFE: 13.59 (4.16 – 28.16); IPF: 71.94 (40.46 - 164); COPD: 25.85 (9.27 – 30.29); p = 0.02]. Conclusions Findings show that CPFE presents different functional and biochemical characteristics than IPF, including higher CV/VC%, CC/TLC% and periostin, whereas mucin-16 was higher in the IPF.

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“…86 Treatment with rituximab could represent a therapeutic option in subjects with aggressive, severe disease 87 ; in most severe cases, lung transplantation may be proposed. The prevalence and clinical implications of small airways involvement in SSc are still to be fully elucidated 88 ; Bonifazi et al recently demonstrated that small airways dysfunction, at IOS assessment, was present in more than 20% of the SSc patients, with a prevalence fivefold higher than in healthy subjects 89 and was significantly associated with worse respiratory-related quality of life. Data from imaging studies further support these findings, as features related to small airways abnormalities (mosaic attenuation pattern and tree-in-bud opacities) were detected in one out of four patients by HRCT chest scans and mostly occurred in the absence of underlying interstitial changes, suggesting that peripheral airways might be an early target of the SSc lung disease rather than a consequence of parenchymal architectural distortion.…”

Section: Ctd-related Bronchiolitismentioning

confidence: 99%

Bronchiolitis and Bronchiolar Disorders

Ravaglia¹,

Poletti

2020

Semin Respir Crit Care Med

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Bronchioles are noncartilaginous small airways with internal diameter of 2 mm or less, located from approximately the eighth generation of purely air conducting airways (membranous bronchioles) down to the terminal bronchioles (the smallest airways without alveoli) and respiratory bronchioles (which communicate directly with alveolar ducts and are in the range of 0.5 mm or less in diameter). Bronchiolar injury, inflammation, and fibrosis may occur in myriad disorders including connective tissue diseases, inflammatory bowel diseases, lung transplant allograft rejection, graft versus host disease in allogeneic stem cell recipients, neuroendocrine cell hyperplasia, infections, drug toxicity (e.g., penicillamine, busulfan), inhalation injury (e.g., cigarette smoke, nylon flock, mineral dusts, hard metals, Sauropus androgynous); idiopathic, common variable immunodeficiency disorder, and a host of other disorders or insults. The spectrum of bronchiolar disorders is wide, ranging from asymptomatic to fatal obliterative bronchiolitis. In this review, we discuss the salient clinical, radiographic, and histological features of these diverse bronchiolar disorders, and discuss a management approach.

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Ventilation distribution and small airway function in patients with systemic sclerosis

Abstract: Ventilation heterogeneity is a frequent finding in SSc patients that is associated with restrictive damage, changes in pulmonary diffusion, and CT patterns. In addition, approximately one-third of the patients presented with findings that were compatible with small airway disease.

Cited by 8 publications

References 27 publications

Ventilation distribution as a contributor to the functional exercise capacity in patients with systemic sclerosis-associated interstitial lung disease without pulmonary hypertension

Ventilation distribution as a contributor to the functional exercise capacity in patients with systemic sclerosis-associated interstitial lung disease without pulmonary hypertension

Periostin and closing volume in combined pulmonary fibrosis and emphysema

Bronchiolitis and Bronchiolar Disorders

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