American journal of respiratory and critical care medicine
-
Am. J. Respir. Crit. Care Med. · Nov 2023
Multicenter Study Clinical TrialImprovement in Lung Clearance Index and Chest CT Scores with Elexacaftor/Tezacaftor/Ivacaftor Treatment in People with Cystic Fibrosis Aged 12 Years and Older - The RECOVER Study.
Rationale: Clinical trials have shown that use of elexacaftor/tezacaftor/ivacaftor (ETI) is associated with improvements in sweat chloride, pulmonary function, nutrition, and quality of life in people with cystic fibrosis (CF). Little is known about the impact of ETI on ventilation inhomogeneity and lung structure. Objectives: RECOVER is a real-world study designed to measure the impact of ETI in people with CF. ⋯ ETI led to improved nutrition and quality of life. People in the F508del/F508del group had significantly lower sweat chloride on ETI treatment compared with the F508del/MF group. Clinical trial registered with www.clinicaltrials.gov (NCT04602468).
-
Am. J. Respir. Crit. Care Med. · Nov 2023
Incorporating Effects of Time Accrued on the Waiting List into Lung Transplantation Survival Models.
Rationale: U. S. lung transplant mortality risk models do not account for patients' disease progression as time accrues between mandated clinical parameter updates. Objectives: To investigate the effects of accrued waitlist (WL) time on mortality in lung transplant candidates and recipients beyond those expressed by worsening clinical status and to present a new framework for conceptualizing mortality risk in end-stage lung disease. ⋯ The multistate model ascribed lower numerical rankings (i.e., higher priority) than CAS for those who died while on the WL (multistate mean; median [interquartile range] ranking at death, 227; 154 [57-334]; CAS median [interquartile range] ranking at death, 329; 162 [11-668]). Patients with interstitial lung disease were more likely to have increasing risk trajectories as a function of time accrued on the WL compared with other lung diagnoses. Conclusions: Incorporating the effects of time accrued on the WL for lung transplant candidates and recipients in donor lung allocation systems may improve the survival of patients with end-stage lung diseases on the individual and population levels.
-
Am. J. Respir. Crit. Care Med. · Nov 2023
Combination of Bronchoalveolar Lavage and CT Differentiates Progressive and Non-Progressive Fibrotic Lung Diseases.
Rationale: Identifying patients with pulmonary fibrosis (PF) at risk of progression can guide management. Objectives: To explore the utility of combining baseline BAL and computed tomography (CT) in differentiating progressive and nonprogressive PF. Methods: The derivation cohort consisted of incident cases of PF for which BAL was performed as part of a diagnostic workup. ⋯ In the validation cohort (n = 290), an increased BAL lymphocyte proportion was associated with a significantly lower probability of disease progression in patients with nonextensive fibrosis or a non-UIP pattern. Conclusions: BAL lymphocytosis is rare in patients with extensive fibrosis or a UIP pattern on CT. In patients without a UIP pattern or with limited fibrosis, a BAL lymphocyte proportion of ⩾25% was associated with a lower likelihood of progression.