Annals of the American Thoracic Society
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The burden of rheumatoid arthritis (RA) and RA-associated interstitial lung disease (RA-ILD) in recent years has not been well characterized. ⋯ The overall RA-related mortality rates are decreasing, however, RA-ILD-related mortality rates remain stable except in age groups 65-84 years. This would suggest that therapies for RA and improvement in the management of other comorbidities have improved the overall outcomes in RA patients, but have had limited effect in the subgroup of patients with RA-ILD.
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Rationale: Clinicians commonly use short-term physiologic markers to assess the benefit of ventilator adjustments. Improved arterial oxygen tension/pressure (PaO2)/fraction of inspired oxygen (FiO2) after ventilator adjustment in acute respiratory distress syndrome is associated with lower mortality. However, as driving pressure (ΔP) reflects lung stress and strain, changes in ΔP may more accurately reflect benefits or harms of ventilator adjustments compared with changes in oxygenation. ⋯ When modeled together in ExPress, ΔΔP (aHR, 1.42; 95% CI, 1.14-1.78) was more strongly associated with mortality than ΔPaO2/FiO2 (aHR, 0.95 per 25 mm Hg increase; 95% CI, 0.90-1.00). Conclusions: Reduced ΔP following protocolized ventilator changes was more strongly and consistently associated with lower mortality than was increased PaO2/FiO2, making ΔΔP more informative about benefit from ventilator adjustments. Our results reinforce the primacy of ΔP, rather than oxygenation, as the key variable associated with outcome.
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Rationale: Lymphangioleiomyomatosis (LAM) is a female-predominant lung disease caused by mutations in the tuberous sclerosis complex (TSC) genes TSC1 and TSC2. Objectives: To examine the association between TSC mutation subtypes and the prevalence of LAM in women with TSC. Methods: Adult women seen at the Cincinnati Children's Hospital Medical Center's TSC clinic were stratified into the following three groups: those with TSC1 mutation, those with TSC2 mutation, and those with no mutation identified (NMI). ⋯ Serum VEGF-D (vascular endothelial growth factor-D) concentrations (median [95% confidence interval]) tended to be higher in patients harboring TSC2 mutations compared with patients with TSC1 mutations (725 pg/ml [612-1,317] vs. 331 pg/ml [284-406]; P = 0.03) and in patients with LAM compared with patients without LAM (725 pg/ml [563-1,609] vs. 429 pg/ml [357-773]; P = 0.02). Conclusions: LAM and angiomyolipomas are more common in women with TSC harboring TSC2 mutations compared with women with TSC1 mutations. Serum VEGF-D is a useful biomarker to suggest the presence of LAM in women with TSC.
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Many patients with obstructive sleep apnea (OSA) experience excessive daytime sleepiness (EDS), which can negatively affect daily functioning, cognition, mood, and other aspects of well-being. Although EDS can be reduced with primary OSA treatment, such as continuous positive airway pressure (CPAP) therapy, a significant proportion of patients continue to experience EDS despite receiving optimized therapy for OSA. This article reviews the pathophysiology and clinical evaluation and management of EDS in patients with OSA. ⋯ Patients who experience residual EDS despite primary OSA therapy may benefit from wake-promoting pharmacotherapy. Agents that inhibit reuptake of dopamine or of dopamine and norepinephrine (modafinil/armodafinil and solriamfetol, respectively) have demonstrated efficacy in reducing EDS and improving quality of life in patients with OSA. Additional research is needed on the effects of wake-promoting treatments on cognition in these patients and to identify individual or disorder-specific responses.
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Randomized Controlled Trial
Frequency and Risk Factors for Reverse Triggering in Pediatric ARDS during Synchronized Intermittent Mandatory Ventilation.
Rationale: Reverse triggering (RT) occurs when respiratory effort begins after a mandatory breath is initiated by the ventilator. RT may exacerbate ventilator-induced lung injury and lead to breath stacking. Objectives: We sought to describe the frequency and risk factors for RT among patients with acute respiratory distress syndrome (ARDS) and identify risk factors for breath stacking. ⋯ Together, these parameters were highly predictive of breath stacking (area under the curve, 0.979). Conclusions: Patients with higher tidal volume who have a set ventilator rate close to their spontaneous respiratory rate are more likely to have RT, which results in breath stacking >25% of the time. Clinical trial registered with ClinicalTrials.gov (NCT03266016).