ERJ Open Research最新文献

Background: Lung or heart-lung transplantation (LT/HLT) for severe pulmonary hypertension (PH) as the primary disease indication carries a high risk of waiting list mortality and post-transplant complications. France and the UK both have coordinated PH patient services but with different referral pathways for accessing LT services.

Methods: We conducted a comparative analysis of adult PH patients listed for LT/HLT in the UK and France.

Results: We included 211 PH patients in France (2006-2018) and 170 in the UK (2010-2019). Cumulative incidence of transplant, delisting and waiting list death within 3 years were 81%, 4% and 11% in France versus 58%, 10% and 15% in the UK (p<0.001 for transplant and delisting; p=0.1 for death). Median non-priority waiting time was 45 days in France versus 165 days in the UK (p<0.001). High-priority listing occurred in 54% and 51% of transplanted patients respectively in France and the UK (p=0.8). Factors associated with achieving transplantation related to recipients' height, male sex, clinical severity and priority listing status. 1-year post-transplant survival was 78% in France and 72% in the UK (p= 0.04).

Conclusion: Access to transplantation for PH patients is better in France than in the UK where more patients were delisted due to clinical deterioration because of longer waiting time. High rates of priority listing occurred in both countries. Survival for those achieving transplantation was slightly better in France. Ensuring optimal outcomes after transplant listing for PH patients is challenging and may involve early listing of higher risk patients, increasing donor lung utilisation and improving allocation rules for these specific patients.

Introduction: Sleepiness while driving is potentially fatal, and it is recommended that a driver who starts to feel tired should stop and have a rest. However, some may use various countermeasures to try to stay alert. We devised a questionnaire that assessed various potential coping strategies that might be used against fatigue and compared them between obstructive sleep apnoea syndrome (OSAS) patients and controls and with sleepiness in general (Epworth Sleepiness Scale (ESS)), specifically while driving (Driving Sleepiness Scale (DSS)) and driving incidents.

Methods: 119 untreated OSAS patients (male 82%, body mass index (BMI) 37±8 kg·m^-2, ESS 14±5, DSS 3±2, oxygen desaturation index (ODI) 39±15) and 105 controls (male 70%, BMI 28±6 kg·m^-2, ESS 4±3, DSS 7±6) matched for age and driving experience were recruited. All completed a questionnaire relating to their experience over the last year, which included sleepiness in general, sleepiness specifically while driving, 10 questions about various coping strategies they might adopt in order to avoid sleepiness and their history of incidents while driving.

Results: As compared to controls, nearly a third of OSAS patients (29.4%) used more than three coping strategies "frequently". OSAS patients who used more than three such strategies had worse ESS (17±4 versus 12±5, p<0.0001); were more likely to feel sleepy while driving (10±8 versus 5±7, p=0.0002) and had more reported accidents (22.85% versus 2.38%, p=0.0002) as compared to OSAS patients who used less than three strategies. There was no difference in patient demographics, severity of OSAS, driving experience or episodes of nodding at the wheel and reported near miss events.

Conclusions: Untreated OSAS patients frequently use certain strategies which could be surrogate markers of sleepiness. Enquiring about such strategies in clinical practice may aid the clinician in identifying the patients who are at risk of driving incidents and to advise appropriately.

Objective: The aim of this study was to create a prognostic instrument for COPD with a multidimensional perspective that includes physical activity (PA). The score also included health status, dyspnoea and forced expiratory volume in 1 s (HADO.2 score).

Methods: A prospective, observational, non-intervention study was carried out. Patients were recruited from the six outpatient clinics of the respiratory service of a single university hospital. The component variables of the HADO.2 score and BODE index were studied, and PA was measured using an accelerometer. The outcomes for the HADO.2 score were mortality and hospitalisations during follow-up and an exploration of the correlation with health-related quality of life at the moment of inclusion in the study.

Results: 401 patients were included in the study and followed up for three years. The HADO.2 score showed good predictive capacity for mortality: C-index 0.79 (0.72-0.85). The C-index for hospitalisations was 0.72 (0.66-0.77) and the predictive ability for quality of life, as measured by R2, was 0.63 and 0.53 respectively for the Saint George's Respiratory Questionnaire and COPD Assessment Test.

Conclusions: There was no statistically significant difference between the mortality predictive capacity of the HADO.2 score and the BODE index. Adding PA to the original BODE index significantly improved the predictive capacity of the index. The HADO.2 score, which includes PA as a key variable, showed good predictive capacity for mortality and hospitalisations. There were no differences in the predictive capacity of the HADO.2 score and the BODE index.

Background: Nintedanib slows progression of lung function decline in patients with progressive fibrosing (PF) interstitial lung disease (ILD) and was recommended for this indication within the United Kingdom (UK) National Health Service in Scotland in June 2021 and in England, Wales and Northern Ireland in November 2021. To date, there has been no national evaluation of the use of nintedanib for PF-ILD in a real-world setting.

Methods: 26 UK centres were invited to take part in a national service evaluation between 17 November 2021 and 30 September 2022. Summary data regarding underlying diagnosis, pulmonary function tests, diagnostic criteria, radiological appearance, concurrent immunosuppressive therapy and drug tolerability were collected via electronic survey.

Results: 24 UK prescribing centres responded to the service evaluation invitation. Between 17 November 2021 and 30 September 2022, 1120 patients received a multidisciplinary team recommendation to commence nintedanib for PF-ILD. The most common underlying diagnoses were hypersensitivity pneumonitis (298 out of 1120, 26.6%), connective tissue disease associated ILD (197 out of 1120, 17.6%), rheumatoid arthritis associated ILD (180 out of 1120, 16.0%), idiopathic nonspecific interstitial pneumonia (125 out of 1120, 11.1%) and unclassifiable ILD (100 out of 1120, 8.9%). Of these, 54.4% (609 out of 1120) were receiving concomitant corticosteroids, 355 (31.7%) out of 1120 were receiving concomitant mycophenolate mofetil and 340 (30.3%) out of 1120 were receiving another immunosuppressive/modulatory therapy. Radiological progression of ILD combined with worsening respiratory symptoms was the most common reason for the diagnosis of PF-ILD.

Conclusion: We have demonstrated the use of nintedanib for the treatment of PF-ILD across a broad range of underlying conditions. Nintedanib is frequently co-prescribed alongside immunosuppressive and immunomodulatory therapy. The use of nintedanib for the treatment of PF-ILD has demonstrated acceptable tolerability in a real-world setting.

Internal normalisation to reference structures on quantitative chest CT imaging (e.g. lung airway dimensions to adjacent vascular dimensions) provides a potential way to standardise image measurements to population characteristics https://bit.ly/3Rh9pnW.

How to identify MAC-PD patients with limited treatment options: an expert consensus https://bit.ly/3QwLQ8T.

Introduction: Pulmonary veno-occlusive disease (PVOD) is a rare and severe subtype of pulmonary arterial hypertension (PAH). Although European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines advise assessing PAH severity at baseline and during follow-up, no existing risk assessment methods have been validated for PVOD. This study aimed to identify prognostic factors, examine the impact of treatment strategies and evaluate risk assessment methods for PVOD patients.

Methods: The study analysed all incident PVOD patients included in the French Pulmonary Hypertension Registry between 2006 and 2021. Survival was assessed based on initial treatment strategy and risk status and compared to a matched (age, sex, pulmonary vascular resistance) PAH group. Six risk assessment methods (number of four low-risk and three noninvasive low-risk variables, ESC/ERS guidelines three-strata and four-strata models, REVEAL 2.0 and Lite 2) were applied at baseline and early follow-up, and their accuracy was compared using Harrell's c-statistic.

Results: Among the 327 included PVOD patients, survival rates at 1, 3 and 5 years were 86%, 50% and 27%, respectively. Multivariate analysis showed that only 6-min walk distance was associated with survival, with no significant difference based on initial treatment strategy. All six risk assessment methods could discriminate mortality risk, and the ESC/ERS four-strata model was the most accurate at both baseline and follow-up (C-index 0.64 and 0.74). PVOD survival rates were consistently lower than PAH when comparing baseline risk status using the ESC/ERS four-strata model.

Conclusion: PVOD is associated with poor outcomes, and initial treatment strategies do not significantly affect survival. Risk assessment methods can be useful in predicting survival for PVOD patients.

Background: Asthma exacerbations reflect disease severity, affect morbidity and mortality, and may lead to declining lung function. Inflammatory endotypes (e.g. T2-high (eosinophilic)) may play a key role in asthma exacerbations. We aimed to assess whether genetic susceptibility underlies asthma exacerbation risk and additionally tested for an interaction between genetic variants and eosinophilia on exacerbation risk.

Methods: UK Biobank data were used to perform a genome-wide association study of individuals with asthma and at least one exacerbation compared to individuals with asthma and no history of exacerbations. Individuals with asthma were identified using self-reported data, hospitalisation data and general practitioner records. Exacerbations were identified as either asthma-related hospitalisation, general practitioner record of asthma exacerbation or an oral corticosteroid burst prescription. A logistic regression model adjusted for age, sex, smoking status and genetic ancestry via principal components was used to assess the association between genetic variants and asthma exacerbations. We sought replication for suggestive associations (p<5×10^-6) in the GERA cohort.

Results: In the UK Biobank, we identified 11 604 cases and 37 890 controls. While no variants reached genome-wide significance (p<5×10^-8) in the primary analysis, 116 signals were suggestively significant (p<5×10^-6). In GERA, two single nucleotide polymorphisms (rs34643691 and rs149721630) replicated (p<0.05), representing signals near the NTRK3 and ABCA13 genes.

Conclusions: Our study has identified reproducible associations with asthma exacerbations in the UK Biobank and GERA cohorts. Confirmation of these findings in different asthma subphenotypes in diverse ancestries and functional investigation will be required to understand their mechanisms of action and potentially inform therapeutic development.

Background: A novel approach to derive prognostic information from echocardiography in pulmonary arterial hypertension (PAH) is to define a phenotype of right heart function combining standard echocardiographic parameters which describe right ventricular pump function and systemic venous congestion. We tested the hypothesis that the combination of advanced strain imaging parameters could yield high prognostic accuracy.

Methods: This was a prospective observational study with a single centre derivation cohort and a second centre validation cohort. The derivation cohort included 49 naive PAH patients who underwent right heart catheterisation and echocardiographic evaluation at baseline and 4-12 months after diagnosis. The validation cohort included 83 prevalent PAH patients who underwent the same examinations at 12 months after diagnosis. We stratified the risk of the derivation cohort according to three models: Model 1, based on haemodynamic parameters; Model 2, based on standard echocardiographic parameters; and Model 3, based on advanced echocardiographic parameters. The median follow-up period was 21 months; the end point of the analysis was clinical worsening.

Results: In the derivation cohort, haemodynamic and echocardiographic parameters obtained at diagnosis were not associated with outcome, whereas a significant association was observed at first reassessment. Model 3 yielded a better predictive accuracy (Harrell's C index 0.832) as compared to Model 2 (Harrell's C index 0.667), and to Model 1 (Harrell's C index 0.713). The validation cohort confirmed the accuracy of Model 3.

Conclusions: A comprehensive assessment of right heart function using right ventricular strain, right atrial reservoir strain and degree of tricuspid regurgitation provides accurate prognostic information in prevalent PAH patients.