Clinical and functional characteristics of individuals with alpha-1 antitrypsin deficiency: EARCO international registry

Background: Alpha-1 antitrypsin deficiency (AATD) is a rare disease that is associated with an increased risk of pulmonary emphysema. The European AATD Research Collaboration (EARCO) international registry was founded with the objective of characterising the individuals with AATD and investigating their natural history.Methods: The EARCO registry is an international, observational and prospective study of individuals with AATD, defined as AAT serum levels1000 individuals with AATD from 15 countries in its first 2 years. Baseline cross sectional data provide relevant information about the clinical phenotypes of the disease, the patterns of functional impairment and factors associated with poor lung function.Pathogenesis and treatment of chronic pulmonary disease

Prognostic impact of acute pulmonary triggers in patients with takotsubo syndrome: new insights from the International Takotsubo Registry

Aims Acute pulmonary disorders are known physical triggers of takotsubo syndrome (TTS). This study aimed to investigate prevalence of acute pulmonary triggers in patients with TTS and their impact on outcomes.Methods and results Patients with TTS were enrolled from the International Takotsubo Registry and screened for triggering factors and comorbidities. Patients were categorized into three groups (acute pulmonary trigger, chronic lung disease, and no lung disease) to compare clinical characteristics and outcomes.Of the 1670 included patients with TTS, 123 (7%) were identified with an acute pulmonary trigger, and 194 (12%) had a known history of chronic lung disease. The incidence of cardiogenic shock was highest in patients with an acute pulmonary trigger compared with those with chronic lung disease or without lung disease (17% vs. 10% vs. 9%, P = 0.017). In-hospital mortality was also higher in patients with an acute pulmonary trigger than in the other two groups, although not significantly (5.7% vs. 1.5% vs. 4.2%, P = 0.13). Survival analysis demonstrated that patients with an acute pulmonary trigger had the worst long-term outcome (P = 0.002). The presence of an acute pulmonary trigger was independently associated with worse long-term mortality (hazard ratio 2.12, 95% confidence interval 1.33-3.38; P = 0.002).Conclusions The present study demonstrates that TTS is related to acute pulmonary triggers in 7% of all TTS patients, which accounts for 21% of patients with physical triggers. The presence of acute pulmonary trigger is associated with a severe in-hospital course and a worse long-term outcome.Cardiolog

Real-world asthma management with inhaler devices in Switzerland-results of the asthma survey.

The aim of the Asthma Survey was to generate insights about the daily practice of physicians with regard to inhaler devices used for treating asthma under real-world conditions in Switzerland. A questionnaire was administered to 605 participating hospital- and practice-based Swiss physicians. Areas of interest were practical aspects of patient education, typical difficulties encountered when prescribing pressurized metered-dose inhalers (pMDI) and dry-powder inhalers (DPI), and reasons for physician preferences. Differences between the German-speaking part of Switzerland (D-CH) and French- and Italian-speaking parts of Switzerland (W-CH) linguistic regions were explored. Datasets from 529 physicians (291 D-CH and 238 W-CH) were suitable for analysis, 342 internists/general practitioners, 177 pulmonologists/allergologists, and 10 other. Approximately 90% of all participants declared being personally involved in providing inhaler device education to their patients. Practice assistants (33.0% vs. 9.2%, P<0.001) and pharmacists (6.9% vs. 19.7%, P<0.001) were more frequently involved in D-CH compared to W-CH. Patient skills with regard to inhalation technique were generally not monitored on a regular basis with only 34.0% of participants ensuring such checks at the scheduled visits. DPIs were overwhelmingly preferred over pMDI. Although the prevalence of typical handling errors was similar with both inhalers in the two regions, pMDIs were used more frequently in W-CH (P<0.001). Real-world asthma management and inhaler preferences differ between D-CH and W-CH. While the importance of patient education is widely acknowledged, inhalation skills monitoring remains suboptimal. The reasons for higher pMDI preference in W-CH compared to D-CH deserve further research

Supplementary Material for: Chronic Obstructive Pulmonary Disease and Cardiac Repolarization: Data from a Randomized Controlled Trial

<b><i>Background:</i></b> Altered cardiac repolarization is a risk factor for sudden cardiac death and seems to be increased in chronic obstructive pulmonary disease (COPD) patients. <b><i>Objective:</i></b> Lung volume reduction surgery (LVRS) has been shown to improve breathing mechanics and lung function in patients with severe COPD and emphysema and possibly also improve altered cardiac repolarization. <b><i>Methods:</i></b> Thirty patients scheduled for LVRS were randomized to LVRS or to the control group. We investigated the treatment effect 3 months after LVRS on measures of cardiac repolarization and dispersion of repolarization (QTc interval, QT dispersion) derived from electrocardiography. Univariable and multivariable analyses were used to identify possible confounders influencing the treatment effect. <b><i>Results:</i></b> LVRS<b> </b>was associated with an improvement in lung function (mean ± SD residual volume/total lung capacity of -9 ± 11% and forced expiratory volume in 1 s of +30 ± 29%). LVRS did not significantly reduce QTc (median -5.3 ms, 95% confidence interval, CI -15.5 to 3.7, p = 0.214) and QT dispersion (median -3.0 ms, 95% CI -13.0 to 7.0, p = 0.536) compared to the control group. No significant association between change in QTc and change in QT dispersion, respectively, and change in possible confounders was found. <b><i>Conclusion:</i></b> LVRS seems to have no effect on cardiac repolarization in patients with COPD. Thus, lung hyperinflation seems not to be a causal mechanism for altered cardiac repolarization in COPD patients

Swiss Recommendations for the Follow-Up and Treatment of Pulmonary Long COVID.

Emerging evidence suggests that long-term pulmonary symptoms and functional impairment occurs in a proportion of individuals following SARS-CoV-2 infection. Although the proportion of affected patients remains to be determined, physicians are increasingly being confronted with patients reporting respiratory symptoms and impairment beyond the acute phase of COVID-19. In face of limited evidence, the Swiss Society for Pulmonology established a working group to address this area of unmet need and formulated diagnostic and treatment recommendations for the care of patients with pulmonary long COVID (LC). The Swiss COVID Lung Study group and Swiss Society for Pulmonology (SSP) formulated 13 questions addressing the diagnosis and treatment of pulmonary LC. A survey within the SSP special interest groups involved in care of LC patients was conducted in Switzerland. A CORE process/Delphi-like process was used to formulate recommendations. Forty experienced pulmonologists replied to the first survey and 22 completed the second follow-up survey. Agreement of ≥70% consensus led to formulation of a recommendation. The participants in the survey reached consensus and formulated a strong recommendation for regarding the following points. Patients hospitalized for COVID-19 should have a pulmonary assessment including pulmonary function tests. Symptomatic subjects affected by COVID-19, including those with mild disease, should benefit from a pulmonary follow-up. Persistent respiratory symptoms after COVID-19 should be investigated by a pulmonary follow-up including plethysmography, diffusion capacity measurement, and blood gases analysis. Individuals having suffered from COVID-19 and who present with persistent respiratory symptoms should be offered a rehabilitation. Additional questions were given moderateor weak recommendations for. The panel did not reach sufficient consensus for pharmacological therapy (e.g., therapy specifically targeting lung fibrosis) to formulate recommendations for LC drug treatment. The formulated recommendations should serve as an interim guidance to facilitate diagnosis and treatment of patients with pulmonary LC. As new evidence emerges, these recommendations may need to be adapted

Supplementary Material for: Predictors of the Overlap Syndrome and Its Association with Comorbidities in Patients with Chronic Obstructive Pulmonary Disease

<b><i>Background:</i></b> The occurrence of both chronic obstructive pulmonary disease (COPD) and obstructive sleep apnea (OSA) in an individual patient has been described as ‘overlap syndrome', which has been associated with poor prognosis. Little is known about the possible predictors of the overlap syndrome and its association with comorbidities contributing to impaired outcome. <b><i>Objectives:</i></b> This study aimed to evaluate the prevalence and possible predictors of the overlap syndrome and its association with comorbidities in a cohort of COPD patients. <b><i>Methods:</i></b> Individuals with COPD (GOLD stages I-IV, risk groups A-D) were recruited from outpatient clinics. Information on age, gender, body mass index (BMI), smoking status, Epworth sleepiness scale (ESS), COPD assessment test, comorbidities, medications and exacerbations in the past year was collected and a spirometry was performed. Participants underwent a nocturnal polygraphy using the ApneaLink™ device at home. An apnea-hypopnea index (AHI) >10 per hour was considered to indicate OSA. <b><i>Results:</i></b> We enrolled 177 COPD patients (112 men) with a mean age of 64 years (range 42-90), of whom 35 (20%) had an ESS score above 10. During nocturnal polygraphy, 33 patients (19%) had evidence of OSA. In multivariate analysis, BMI and pack years were positively associated with AHI, independent of other significant AHI determinants from univariate analysis. Arterial hypertension and diabetes were more common in patients with the overlap syndrome. <b><i>Conclusions:</i></b> Almost 20% of COPD patients also have OSA. BMI and smoking history seem to be predictors of the overlap syndrome, and these patients may be more often affected by hypertension and diabetes

Nighttime features derived from topic models for classification of patients with COPD

Nighttime symptoms are important indicators of impairment for many diseases and particularly for respiratory diseases such as chronic obstructive pulmonary disease (COPD). The use of wearable sensors to assess sleep in COPD has mainly been limited to the monitoring of limb motions or the duration and continuity of sleep. In this paper we present an approach to concisely describe sleep patterns in subjects with and without COPD. The methodology converts multimodal sleep data into a text representation and uses topic modeling to identify patterns across the dataset composed of more than 6000 assessed nights. This approach enables the discovery of higher level features resembling unique sleep characteristics that are then used to discriminate between healthy subjects and those with COPD and to evaluate patients' disease severity and dyspnea level. Compared to standard features, the discovered latent structures in nighttime data seem to capture important aspects of subjects sleeping behavior related to the effects of COPD and dyspnea

Idiopathic Pulmonary Fibrosis in Switzerland: Diagnosis and Treatment.

Idiopathic pulmonary fibrosis (IPF) is a severe progressive and irreversible lung disease. Novel antifibrotic drugs that slow disease progression are now available. However, many issues regarding patient management remain unanswered, such as the choice between available drugs, their use in particular subgroups and clinical situations, time of treatment onset, termination, combination or switch, or nonpharmacologic management. To guide Swiss respiratory physicians in this evolving field still characterized by numerous areas of uncertainty, the Swiss Working Group for interstitial and rare lung diseases of the Swiss Respiratory Society provides a position paper on the diagnosis and treatment of IPF