Feasibility and acceptability of remotely monitoring spirometry and pulse oximetry as part of interstitial lung disease clinical care: a single arm observational study.

BACKGROUND: Remote monitoring of patient-recorded spirometry and pulse oximetry offers an alternative approach to traditional hospital-based monitoring of interstitial lung disease (ILD). Remote spirometry has been observed to reasonably reflect clinic spirometry in participants with ILD but remote monitoring has not been widely incorporated into clinical practice. We assessed the feasibility of remotely monitoring patients within a clinical ILD service. METHODS: Prospective, single-arm, open-label observational multi-centre study (NCT04850521). Inclusion criteria included ILD diagnosis, age ≥ 18 years, FVC ≥ 50% predicted. 60 participants were asked to record a single spirometry and oximetry measurement at least once daily, monitored weekly by their local clinical team. Feasibility was defined as ≥ 68% of participants with ≥ 70% adherence to study measurements and recording measurements ≥ 3 times/week throughout. RESULTS: A total of 60 participants were included in the analysis. 42/60 (70%) were male; mean age 67.8 years (± 11.2); 34/60 (56.7%) had idiopathic pulmonary fibrosis (IPF), Median ILD-GAP score was 3 (IQR 1-4.75). Spirometry adherence was achieved for ≥ 70% of study days in 46/60 participants (77%) and pulse oximetry adherence in 50/60 participants (83%). Recording ≥ 3 times/week every week was provided for spirometry in 41/60 participants (68%) and pulse oximetry in 43/60 participants (72%). Mean difference between recent clinic and baseline home spirometry was 0.31 L (± 0.72). 85.7% (IQR 63.9-92.6%) home spirometry attempts/patient were acceptable or usable according to ERS/ATS spirometry criteria. Positive correlation was observed between ILD-GAP score and adherence to spirometry and oximetry (rho 0.24 and 0.38 respectively). Adherence of weekly monitoring by clinical teams was 80.95% (IQR 64.19-95.79). All participants who responded to an experience questionnaire (n = 33) found remote measurements easy to perform and 75% wished to continue monitoring their spirometry at the conclusion of the study. CONCLUSION: Feasibility of remote monitoring within an ILD clinical service was demonstrated over 3 months for both daily home spirometry and pulse oximetry of patients. Remote monitoring may be more acceptable to participants who are older or have more advanced disease. TRIAL REGISTRATION: clinicaltrials.gov NCT04850521 registered 20th April 2021.

Host predisposition by endogenous Transforming Growth Factor-beta1 overexpression promotes pulmonary fibrosis following bleomycin injury.

BACKGROUND: Idiopathic Pulmonary Fibrosis (IPF) is a progressive diffuse disease involving the lung parenchyma. Despite recent advances, the molecular mechanisms of the initiation and progression of this disease remain elusive. Previous studies have demonstrated TGFbeta1 as a key effector cytokine in the development of lung fibrosis. METHODS: In this study we have used a transgenic mouse based strategy to identify the effect of overexpression of this key effector mediator on the development of pulmonary fibrosis in response to exogenous injury. We bred two lines (line 25 and 18) of transgenic mice (Tr+) that overexpressed active TGFbeta1. Three-month old transgenic and wild type mice were subsequently wounded with intraperitoneal bleomycin. Mice were sacrificed at 6 weeks post-bleomycin and their lungs analysed histologically and biochemically. RESULTS: The severity of lung fibrosis was significantly greater in the Tr+ mice compared to the wild type mice. Using an oligonucleotide microarray based strategy we identified discrete patterns of gene expression contributing to TGFbeta1 associated pulmonary fibrosis. CONCLUSION: This data emphasises the importance of a host predisposition in the form of endogenous TGFbeta1, in the development of pulmonary fibrosis in response to an exogenous injury.

Bronchiolitis obliterans syndrome in single lung transplant recipients--patients with emphysema versus patients with idiopathic pulmonary fibrosis.

Bronchiolitis obliterans syndrome (BOS) after lung transplantation is a disease of small airways that is currently graded according to a decline in forced expiratory volume in 1 second (FEV(1)) even in single lung transplant recipients in whom native diseased lung may influence lung physiology. The aim of this study was to evaluate the comparative changes in lung function and survival following the onset of BOS in patients with emphysema and patients with idiopathic pulmonary fibrosis (IPF) who have undergone single lung transplantation. We analyzed data from 31 single lung transplant recipients with emphysema and 25 with IPF who were at risk of BOS. There was no difference in the incidence of BOS between the 2 groups (10 patients with emphysema and 6 patients with IPF), but after the onset of BOS the patients with emphysema had a significantly greater median survival (18 months vs 8 months) despite a poorer mean FEV(1) (1.26 liter, 45% predicted vs 2.11 liter, 67% predicted) compared with the IPF group (p < 0.05) and this difference in lung function persisted at death (0.8 liter, 30% predicted vs 1.65 liter, 51% predicted) (p < 0.05). In summary the native lung physiology appears to influence lung function and therefore survival, and this may indicate that the classification of BOS should include disease-specific characteristics.