Patients with bronchiectasis feature considerable symptom burden and reduced health-related quality of life (QOL). We provide the psychometric validation of the German translation of the disease-specific Quality of Life Questionnaire-Bronchiectasis (QOL-B), version 3.1, using baseline data of adults consecutively enrolled into the prospective German bronchiectasis registry PROGNOSIS. Overall, 904 patients with evaluable QOL-B scores were included. We observed no relevant floor or ceiling effects. Internal consistency was good to excellent (Cronbach’s α ≥0.73 for each scale). QOL-B scales discriminated between patients based on prior pulmonary exacerbations and hospitalizations, breathlessness, bronchiectasis severity index, lung function, sputum volume, Pseudomonas aeruginosa status and the need for regular pharmacotherapy, except for Social Functioning, Vitality and Emotional Functioning scales. We observed moderate to strong convergence between several measures of disease severity and QOL-B scales, except for Social and Emotional Functioning. Two-week test-retest reliability was good, with intraclass correlation coefficients ≥0.84 for each scale. Minimal clinical important difference ranged between 8.5 for the Respiratory Symptoms and 14.1 points for the Social Functioning scale. Overall, the German translation of the QOL-B, version 3.1, has good validity and test-retest reliability among a nationally representative adult bronchiectasis cohort. However, responsiveness of QOL-B scales require further investigation during registry follow-up.

Background: Small-cell lung cancer (SCLC) is a highly aggressive tumor, and overall survival (OS) remains poor despite intensive efforts to develop new treatment strategies. In second line, topotecan is the only approved drug, with a median OS of 5.9 months. However, real-world SCLC patients are often in worse condition and harbor more comorbidities than study populations. Therefore, the real-world performance of topotecan may differ from that seen in studies. Here, we analyzed outcomes of SCLC patients receiving topotecan and identified predictive and prognostic markers. Patients and Methods: We retrospectively analyzed 44 consecutive SCLC patients receiving topotecan between 2015 and 2022. We analyzed baseline characteristics (age, ECOG-PS, topotecan cycles, and dosage) and pre-treatment blood values (LDH, CRP, sodium) as well as prognostic scores (neutrophil/lymphocyte ratio (NLR), thrombocyte/lymphocyte ratio (TLR), Glasgow Prognostic Score, prognostic nutritional score, systemic inflammation index (SII), and the prognostic index) extracted from electronic patients’ charts to identify predictive and prognostic markers. Results: In our cohort, mPFS and mOS were only 1.9 and 5.6 months, respectively. Gender, ECOG-PS, active brain metastases, NLR, GPS, PNI, and SII significantly influenced PFS and OS in univariate analysis. ECOG-PS (p > 0.001), active brain metastases (p = 0.001), and SII (p = 0.008) were significant independent prognostic variables in a multivariate COX regression model. Selecting patients by these three markers achieved an mPFS of 5.7 months and thus increased the mPFS three-fold. Patients not meeting all criteria had an mPFS of 1.8 months (p = 0.006). Patients identified by prognostic markers had an mOS of 9.1 months (p = 0.002). Conclusions: The efficacy of topotecan in SCLC real-world patients is poor, indicating that many patients were treated without any benefit. Easy-to-obtain markers can predict response and treatment efficacy and should therefore be validated in larger cohorts to identify patients who are more likely to benefit from topotecan.

Background: In line with its increasing prevalence, pulmonary Mycobacterium avium complex (MAC) disease (MAC-PD) gives rise to substantial healthcare costs. However, there is only limited information on the costs of intersectoral reimbursement. Objectives: Inpatient and outpatient costs for diagnosing and treating pulmonary MAC-PD in Germany in accordance with standard international guidelines were calculated and their potential effects on MAC disease management in Germany were determined. Methods: Hospitalization costs were calculated by using the German diagnosis related group (G-DRG) browser, with and without inclusion of the diseases most often associated with M. avium. Separated by drug macrolide susceptibility and severity of MAC-PD, the direct medical costs of suitable therapies in the outpatient setting were determined by Monte-Carlo simulation, including all conceivable options. Results: According to our simulation, the weighted mean cost of outpatient treatment over 14 or 18 months, in either case followed by a post-treatment monitoring over 12 months, amounts to €8675.22 (95% confidence interval [CI] €8616.17 to €8734.27). Of that amount, the revenue for outpatient doctors´ services, dependent on treatment duration, is low, ranging between €894.79 (10.3%) and €979.42 (11.3%), accordingly. Mean drug costs for MAC-PD patients amount to €6130.25 [95% CI €6073.52 to €6186.98], i.e., more than two third (70.7%) of the total outpatient costs. In contrast, the non-surgical reimbursement for a hospital stay of up to 14 days is €3321.64. Hospital reimbursement does not increase in cases of complications (a higher number and/or challenging type of associated diseases), but it is fully paid even in cases that require as few as 2 days of hospitalization. Conclusion: The imbalance between well-rewarded hospital care and the low reimbursement for long-term treatment of MAC-PD outpatients may induce inappropriate disease management. In order to arrive at properly integrated care of MAC-PD patients in Germany, measures such as better incentives for physicians in the outpatient setting and a targeted use of resources in hospitals are required. Reimbursed, periodic case conferences between outpatient physicians and experts in hospitals as well as preventive short-term checks of MAC-PD patients in specialty clinics may promote cross-sector cooperation and improve overall treatment quality. Nationwide pilot studies are required to gain evidence on the effectiveness of the new approach.

Objectives: To investigate the role of blood eosinophils in predicting PH in end-stage lung disease. Methods: We conducted a retrospective study of adults with CF, COPD, and ILD who underwent RHC during lung transplant evaluations (2010–2022). Patients were classified by the 2022 ECS/ERS PH guidelines with pulmonary function and laboratory tests, including hemograms. The eosinophil threshold was set at 0.30 G/L. Results: We analyzed 663 patients (n = 89 CF, n = 294 COPD, and n = 280 ILD). Severe PH was more common in ILD (16%) than in CF (4%) and COPD (7%) (p = 0.0002), with higher eosinophil levels in ILD (p = 0.0002). No significant correlation was found between eosinophil levels and hemodynamic parameters (PAPm, PVR, and CI) across CF, COPD, and ILD (PAPm: p = 0.3974, p = 0.4400 and p = 0.2757, respectively; PVR: p = 0.6966, p = 0.1489 and p = 0.1630, respectively; CI: p = 0.9474, p = 0.5705 and p = 0.5945, respectively), nor was a correlation observed in patients not receiving OCS. Linear regression analysis confirmed the lack of association (PAPm: p = 0.3355, p = 0.8552 and p = 0.4146, respectively; PVR: p = 0.6924, p = 0.8935 and p = 0.5459, respectively; CI: p = 0.4260, p = 0.9289 and p = 0.5364, respectively), controlling for 6-MWD, Nt-proBNP, and ICS/OCS dosages. ROC analysis indicated eosinophils were ineffective in distinguishing PH severity levels across these diseases (AUC 0.54, 0.51, and 0.53, respectively). The analysis of eosinophil levels measured 18 ± 6 months prior to baseline found no predictive correlation with the presence of PH either. Eosinophil levels did not differ significantly among PH groups, but eosinophilic COPD was linked to more unclassified PH, higher CO, and greater lung volumes than non-eosinophilic COPD. Conclusions: In our cohort of end-stage CF, COPD, and ILD patients, blood eosinophilia did not predict the presence of PH but was associated with hemodynamic parameters and lung volumes in COPD.

Extract Williams-Campbell Syndrome (WCS) is a rare pulmonary disorder encompassing aspects of both bronchiectasis and obstructive lung disease. First described by Williams and Campbell in 1960 [1], this syndrome is characterised by the absence of bronchial cartilage, leading to bronchiectasis and recurrent respiratory infections [2, 3]. Due to its rarity, WCS poses significant challenges in terms of diagnosis and management.