[S3 Guideline: Treating Chronic Respiratory Failure with Non-invasive Ventilation]. / S3-Leitlinie: Nichtinvasive Beatmung als Therapie der chronischen respiratorischen Insuffizienz.

The S3 guideline on non-invasive ventilation as a treatment for chronic respiratory failure was published on the website of the Association of the Scientific Medical Societies in Germany (AWMF) in July 2024. It offers comprehensive recommendations for the treatment of chronic respiratory failure in various underlying conditions, such as COPD, thoraco-restrictive diseases, obesity-hypoventilation syndrome, and neuromuscular diseases. An important innovation is the separation of the previous S2k guideline dating back to 2017, which included both invasive and non-invasive ventilation therapy. Due to increased scientific evidence and a significant rise in the number of affected patients, these distinct forms of therapy are now addressed separately in two different guidelines.The aim of the guideline is to improve the treatment of patients with chronic respiratory insufficiency using non-invasive ventilation and to make the indications and therapy recommendations accessible to all involved in the treatment process. It is based on the latest scientific evidence and replaces the previous guideline. This revised guideline provides detailed recommendations on the application of non-invasive ventilation, ventilation settings, and the subsequent follow-up of treatment.In addition to the updated evidence, important new features of this S3 guideline include new recommendations on patient care and numerous detailed treatment pathways that make the guideline more user-friendly. Furthermore, a completely revised section is dedicated to ethical issues and offers recommendations for end-of-life care. This guideline is an important tool for physicians and other healthcare professionals to optimize the care of patients with chronic respiratory failure. This version of the guideline is valid for three years, until July 2027.

Impact of the COVID-19 Pandemic on Home Mechanical Ventilation in Germany: A Descriptive Observational Study.

INTRODUCTION: Over the last decade, the number of patients receiving home mechanical ventilation (HMV) has increased significantly, which has led to a limited availability of specialist centres, not least due to the scarcity of healthcare professionals. This situation was exacerbated by the COVID-19 pandemic. It is therefore assumed that the repurposing of resources has led to an aggravated change in the healthcare structure in HMV. METHODS: This descriptive observational study analysed the Operation and Procedure Classification Codes for patients receiving HMV from 2008 to 2022. The data were provided by the Federal Statistical Office of Germany. Data were additionally analysed with respect to geographical distribution and ventilation status. RESULTS: A total of 737,770 datasets were analysed (mean age in 2020: 66.5 years). There was a steady increase in HMV initiations (+6%) and controls (+9%) per year before the pandemic (2008-2019). Patient admissions during the pandemic revealed a 28% decrease, with the largest decrease in invasive ventilation (IV) follow-up visits (2019: 3,053; 2020: 2,199; -39%), while the number of IV initiations remained stable. There was a 19% decrease in the number of non-IV initiations in 2020 (16,919 vs. 14,227) and a 32% decrease in the number of follow-ups (45,812 vs. 34,813) in comparison with 2019. CONCLUSION: The pandemic has led to a significant decline of inpatient admissions for patients receiving HMV. This decline was most pronounced in the first year of the pandemic. Control visits in particular did not reach the pre-pandemic level. This is an indication of the ongoing change in the healthcare landscape as a result of the pandemic.

Adherence-enhancing interventions for pharmacological and oxygen therapy in patients with COPD: a systematic review and component network meta-analyses.

INTRODUCTION: Adherence to COPD management strategies is complex, and it is unclear which intervention may enhance it. OBJECTIVES: We aim to evaluate the effectiveness of adherence-enhancing interventions, alone or compared to interventions, for patients with COPD. METHODS: This review comprises a component network meta-analysis with a structured narrative synthesis. We searched MEDLINE, Embase, CENTRAL, CINAHL and trial registries on 9 September 2023. We included controlled studies that explored adherence in patients with COPD. Two review authors independently performed the study selection, data extraction and the risk of bias assessment. We involved patients with COPD in developing this systematic review through focus group interviews and displayed the findings in pre-designed logic models. RESULTS: We included 33 studies with 5775 participants. We included 13 studies in the component network meta-analysis that explored adherence. It was mainly assessed through questionnaires. As a continuous outcome, there was a tendency mainly for education (standardised mean difference 1.26, 95% CI 1.13-1.38, very low certainty of evidence) and motivation (mean difference 1.85, 95% CI 1.19-2.50, very low certainty of evidence) to improve adherence. As a dichotomous outcome (e.g. adherent/non-adherent), we found a possible benefit with education (odds ratio 4.77, 95% CI 2.25-10.14, low certainty of evidence) but not with the other components. We included six studies that reported quality of life in the component network meta-analysis. Again, we found a benefit of education (mean difference -9.70, 95% CI -10.82- -8.57, low certainty of evidence) but not with the other components. CONCLUSIONS: Education may improve adherence and quality of life in COPD patients. Patient focus group interviews indicated that interventions that strengthen patients' self-efficacy and help them to achieve individual goals are the most helpful.

Care pathways versus usual care for chronic obstructive pulmonary disease (COPD).

OBJECTIVES: This is a protocol for a Cochrane Review (intervention). The objectives are as follows: To assess the effects of care pathways (CPs) compared to usual care/no CPs for people with chronic obstructive pulmonary disease (COPD).

[Long-term health-related quality of life of patients after prolonged weaning depending on weaning status]. / Lebensqualität im Langzeitverlauf nach prolongiertem Weaning in Abhängigkeit vom Weaning-Status.

INTRODUCTION: Long-term outcome in patients with prolonged weaning is known to be impaired, particularly against the background of their weaning status; however, data on their health-related quality of life (HRQL) are sparse. METHODS: HRQL was measured in patients with prolonged weaning using the Severe Respiratory Insufficiency Questionnaire (SRI). RESULTS: Overall, 39 out of 83 patients with prolonged weaning filled in the SRI questionnaire. The median interval between discharge from hospital and HRQL assessment was 3.5 years (IQR 2.4-4.5 years). In the total group, the median SRI summary score was 56.4 (IQR 38.8-73.5). Patients with unsuccessful weaning and subsequent invasive home mechanical ventilation (N=15) had worse HRQL as estimated from the SRI summary score when compared to those with successful weaning both without (n=13) and with subsequent long-term non-invasive ventilation (NIV) (n=11); Kruskal-Wallis-Test: H (2, n=39) = 7,875446; P=0.0195. Statistically significant differences indicating worse HRQL in patients with invasive home mechanical ventilation were particularly evident in the following SRI subscales: Social relationships (P=0.0325), Anxiety (P=0.0096), and Psychological well-being (P=0.0079). CONCLUSIONS: HRQL is substantially impaired in patients with unsuccessful prolonged weaning and subsequent invasive home mechanical ventilation compared to those with successful prolonged weaning. Further studies incorporating higher case numbers are needed to assess other conditions potentially affecting HRQL in patients with prolonged weaning.

A clinical phenotype of VEXAS syndrome with pleural effusion, infiltrates, and systemic inflammation in a 76-year-old patient: a case report.

INTRODUCTION: VEXAS syndrome, characterized by a UBA1 gene mutation, is a rare and severe systemic inflammatory disease predominantly affecting men. Since its initial description in 2020, it has been noted for its broad clinical phenotype and frequent misdiagnosis. CASE PRESENTATION: A 76-year-old Caucasian male patient diagnosed with VEXAS syndrome is presented in this case report. He presented with typical symptoms including pulmonary manifestations (infiltrates and effusions), systemic inflammation, and haematological abnormalities. The diagnosis was challenging due to the disease's heterogeneous presentation, often resembling autoimmune or haematological diseases. This patient's case featured ground-glass opacities and pleural effusions, underlining the significant pulmonary involvement seen in 50-67% of VEXAS patients. His condition was further complicated by recurrent fever and systemic inflammation affecting multiple organs. CONCLUSION: VEXAS syndrome demands an aggressive treatment approach due to its high mortality rate and refractory nature. This case underscores the importance of including VEXAS syndrome in differential diagnoses, particularly for patients with systemic inflammation and pulmonary symptoms, and calls for multidisciplinary management and extensive research to understand its full range of clinical phenotypes.

Clinical relevance of positively determined myositis antibodies in rheumatology: a retrospective monocentric analysis.

BACKGROUND: The increased availability of myositis autoantibodies represents new possibilities and challenges in clinical practice (Lundberg IE, Tjärnlund A, Bottai M, Werth VP, Pilkington C, de Visser M, et al. 2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. Ann Rheum Dis. 2017;76:1955-64. https://doi.org/10.1136/annrheumdis-2017-211468 .). The aim of this study was to perform a retrospective data analysis of patient cases with positive myositis autoantibodies to analyse their significance in routine rheumatology practice. METHODS: A monocentric analysis of all the orders used to determine myositis autoantibodies from July 2019 to May 2022 in the Department of Rheumatology, Krankenhaus Porz am Rhein, Cologne, Germany, was carried out. RESULTS: In the defined time interval, a total of 71,597 laboratory values for the antibodies mentioned above were obtained. A total of 238 different positive autoantibodies ​​were detected in 209 patients. Idiopathic inflammatory myopathy was diagnosed in 37 patients (18%), and inflammatory rheumatic diseases other than idiopathic inflammatory myopathy were diagnosed in 90 patients (43%). No inflammatory rheumatic disease was diagnosed in 82 patients (39%). General clusters of clinical manifestations were observed. CONCLUSIONS: In our cohort, we were able to show that a relevant proportion of patients with positive myositis antibodies did not have idiopathic inflammatory myopathies or inflammatory rheumatic diseases. This finding indicates the importance of myositis autoantibodies in this group of patients. However, further studies on the course of symptoms and examination results in patients without inflammatory rheumatic diseases and with positive myositis antibodies are necessary.

Deventilation Syndrome in COPD Patients Receiving Long-Term Home Noninvasive Ventilation: A Systematic Scoping Review.

The treatment of patients with COPD and chronic hypercapnic respiratory failure using noninvasive ventilation (NIV) is well established. A "deventilation syndrome" (DVS) has been described as acute dyspnea after cessation of NIV therapy. A systematic scoping review reporting according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-ScR) searching Embase was conducted in September 2021. A final manual search followed in February 2023. Literature synthesis was blinded using Rayyan by three different reviewers. A total of 2,009 studies were screened. Five studies met the eligibility criteria. Four articles presented original data. Three articles examined potential treatment options. Three studies were prospective; none were randomized. A total of 122 patients were included. DVS was defined differently in all studies. Seventy-four patients were identified to suffer from DVS (48 controls). Patients were evaluated by blood gas analysis, transcutaneous TcCO2 measurement, spirometry, whole-body plethysmography, respiratory muscle assessments, diaphragmatic electromyography, ultrasound, 6-min walk test, polysomnography, and questionnaires. Treatment approaches studied were minimization of "patient-ventilator asynchrony" (PVA) and use of pursed- lip breathing ventilation. Pathophysiological mechanisms discussed were PVA, high inspiratory positive airway pressure, hyperinflation, respiratory muscle impairment, and increased respiratory rates. Compared with controls, patients with DVS appeared to suffer from more severe airway obstruction, hyperinflation, and PaCO2 retention; worse exercise test scores; and poorer quality of life. The available evidence does not allow for definite conclusions about pathophysiological mechanisms, ethology, or therapeutic options. Future studies should focus on a consistent definition and possible pathomechanisms.

[Home mechancial ventilation: quality of life and the final stage of life]. / Außerklinische Beatmung: Lebensqualität und Lebensende.

The evaluation of health-related quality of life (HRQL) has gained importance in recent years. Disease-specific questionnaires are available for respiratory insufficiency, which enable the assessment of HRQL. The Severe Respiratory Insufficiency (SRI) questionnaire, which was specially developed for patients with respiratory insufficiency, is ideal for quantifying the quality of life in patients receiving home mechanical ventilation (HMV). Studies using the SRI questionnaire demonstrated that the physical functioning of patients with chronic lung diseases of various etiologies is significantly impaired, but frequently without significant impairment of psychological well-being. Therefore, severity of the disease and HRQL do not necessarily correlate with each other.Both invasive and non-invasive mechanical ventilation can improve quality of life. Co-morbidity, and above all advanced age, have the highest negative predictive value with regard to in-hospital mortality and weaning failure. The number of patients older than 80 years who remain dependent on invasive HMV after prolonged weaning in Germany is increasing significantly. High dependence on invasive HMV is often associated with a loss of quality of life and autonomy. Thus, ethical issues of the continued treatment of ventilated patients at the end of life are discussed increasingly.After weaning failure, the indication for invasive HMV should be critically examined and should focus on potentially severely reduced quality of life and poor prognosis, as well as the patient's wishes. If previously agreed treatment goals can no longer be achieved during the course of invasive HMV, changing the therapy goals should be discussed within the clinical team, with the patient, their relatives and, if necessary, with legal representatives.In order to avoid overtreatment and unnecessary patient suffering during invasive HMV, advanced care planning should be started as early as possible and if necessary accompanied by palliative medical measures.

Ambulatory Long-Term Oxygen Therapy in Patients with Severe COPD: A Randomized Crossover Trial to Compare Constant-Minute-Volume and Constant-Bolus Systems.

Background and Methods: Constant-minute-volume and constant-bolus devices serve as two different means of portable oxygen conservation. A prospective randomised crossover study was conducted in COPD GOLD IV patients to investigate the effect of these two devices on dyspnea, oxygenation and 6-minute walking test (6MWT) distance. The primary endpoint was the final operating level required (operating level range 1-5 for both devices) by either device to meet the success criteria for mobile oxygen therapy, as outlined in the British Thoracic Society guidelines (SpO2 ≥90% throughout 6MWT; ≥10% increase in walking distance from baseline; improvement in BORG of at least 1 point from baseline). Results: Twenty-five patients were enrolled in the study and randomly assigned to one of two sequences involving the use of each type of portable oxygen conservation device. 14 female, 67.9 years (±7.8); FEV1: 27.3%pred. (±8.4); PaO2 at rest without oxygen: 50.3mmHg (±5.9). For both systems, 24/25 patients (96%) were successfully recruited. The mean operating-level difference when success criteria were met was -0.58 in favor of the constant bolus device (95% CI: -0.88 to -0.28, P <0.001). Secondary endpoints (walking distance, respiratory rate and BORG dyspnea) showed no statistically significant or clinically relevant differences. An algorithm created especially for this study showed a high success rate in terms of titration for the required operating level. Conclusion: Both portable oxygen-conserving devices met the success criteria in 96% of patients in the 6MWT when they were titrated to the correct level. The constant-bolus device required a significantly lower operating level to achieve the success criteria, hereby reducing energy consumption. Individual titration of the respective device is recommended, which can be facilitated by the novel titration algorithm described here.