Ventilation distribution and small airway function in patients with systemic sclerosis
Bruno Rangel Antunes da Silva,Rogério Rufino,Cláudia Henrique da Costa,Verônica Silva Vilela,Roger A. Levy,Agnaldo José Lopes +5 more
TLDR
Ventilation heterogeneity is a frequent finding in SSc patients that is associated with restrictive damage, changes in pulmonary diffusion, and CT patterns, and in addition, approximately one-third of the patients presented with findings that were compatible with small airway disease.Abstract:
Background Despite the importance of traditional pulmonary function tests (PFTs) in managing systemic sclerosis (SSc), many patients with pulmonary disease diagnosed by computed tomography (CT) present with normal PFTs. Objective To evaluate the efficacy of the nitrogen single-breath washout (N2SBW) test in diagnosing SSc and to correlate N2SBW parameters with the PFT indexes used in the follow-up of these patients, clinical data, and CT findings. Methods Cross-sectional study in which 52 consecutive SSc patients were subjected to spirometry, body plethysmography, analysis of the diffusing capacity for carbon monoxide (DLCO), analysis of respiratory muscle strength, N2SBW testing, and CT analysis. Results Twenty-eight patients had a forced vital capacity (FVC) that was 120% of the predicted value, while 15 patients had a closing volume/vital capacity (CV/VC) that was >120% of the predicted value. A significant difference in Phase III slopeN2SBW was observed when the patients with predominant traction bronchiectasis and honeycombing were compared to the patients with other CT patterns (p Conclusion 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.read more
Citations
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Pulmonary Manifestations of Systemic Sclerosis and Mixed Connective Tissue Disease.
TL;DR: Treatment with either cyclophosphamide or mycophenolate has been shown to delay disease progression, whereas rituximab and lung transplantation are reserved for refractory cases.
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Increased prevalence of small airways dysfunction in patients with systemic sclerosis as determined by impulse oscillometry.
Martina Bonifazi,Nicola Sverzellati,Eva Negri,Giovanni Pomponio,Valeria Seletti,Matteo Bonini,Paolo Fraticelli,Luca Paolini,Massimo Mattioli,Matteo Franchi,Irene Tramacere,Venerino Poletti,Carlo La Vecchia,Stefano Gasparini,Armando Gabrielli +14 more
TL;DR: The hypothesis that small airway dysfunction might be a feature of SSc-related lung involvement is strengthened, providing the first data on its significant impact on respiratory-related quality of life.
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Prevalence and clinical correlates of small airway obstruction in patients with systemic sclerosis.
Predrag Ostojic,Marina Vujovic +1 more
TL;DR: Small airway obstruction in patients with systemic sclerosis can be considered a clinical feature of undiagnosed obstructive lung disease, if isolated or associated with largeAirway obstruction, especially in tobacco smokers, and may indicate a possible prominent bronchiolar involvement within SSc-related interstitial lung disease.
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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.
TL;DR: The impact of ventilation distribution heterogeneity assessed by the nitrogen single-breath washout (N2SBW) test and peripheral muscle dysfunction on the exercise capacity in patients with SSc-ILD and limited involvement of the pulmonary parenchyma was evaluated.
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Bronchiolitis and Bronchiolar Disorders.
TL;DR: The salient clinical, radiographic, and histological features of these diverse bronchiolar disorders, ranging from asymptomatic to fatal obliterative Bronchiolitis are discussed.
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