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Purpose Coronavirus disease 2019 (COVID-19) caused by severe acute respiratory coronavirus 2 (SARS-CoV-2) has spread around the world. Differentiation between pure viral COVID-19 pneumonia and secondary infection can be challenging. In patients with elevated C-reactive protein (CRP) on admission physicians often decide to prescribe antibiotic therapy. However, overuse of anti-infective therapy in the pandemic should be avoided to prevent increasing antimicrobial resistance. Procalcitonin (PCT) and CRP have proven useful in other lower respiratory tract infections and might help to differentiate between pure viral or secondary infection. Methods We performed a retrospective study of patients admitted with COVID-19 between 6th March and 30th October 2020. Patient background, clinical course, laboratory findings with focus on PCT and CRP levels and microbiology results were evaluated. Patients with and without secondary bacterial infection in relation to PCT and CRP were compared. Using receiver operating characteristic (ROC) analysis, the best discriminating cut-off value of PCT and CRP with the corresponding sensitivity and specificity was calculated. Results Out of 99 inpatients (52 ICU, 47 Non-ICU) with COVID-19, 32 (32%) presented with secondary bacterial infection during hospitalization. Patients with secondary bacterial infection had higher PCT (0.4 versus 0.1 ng/mL; p = 0.016) and CRP (131 versus 73 mg/L; p = 0.001) levels at admission and during the hospital stay (2.9 versus 0.1 ng/mL; p p p p Conclusion PCT and CRP measurement on admission and during the course of the disease in patients with COVID-19 may be helpful in identifying secondary bacterial infections and guiding the use of antibiotic therapy.

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 and aims Bacterial infections are common in patients with decompensated liver cirrhosis and a leading cause of death. Reliable data on antibiotic resistance are required to initiate effective empiric therapy. We here aim to assess the antimicrobial resistance profile of bacteria among patients with liver cirrhosis and infection. Methods Overall, 666 cirrhotic patients admitted to Hannover Medical School between January 2012 and April 2018 with ascites were assessed for bacterial infection. In case of infection, bacteria cultured from microbiological specimens of ascites, blood or urine were identified and analyzed for resistances against common antibiotic agents. Furthermore, analyses compared two periods of time and community-acquired vs. nosocomial infections. Results In 281 patients with infection, microbiological sampling was performed and culture-positive results were obtained in 56.9%. Multidrug-resistant (MDR)-bacteria were found in 54 patients (19.2%). Gram-positive organisms were more common (n = 141/261, 54.0%) and detected in 116/192 culture-positive infections (60.4%). Comparing infections before and after 2015, a numerical decline for MDR-bacteria (23.8% vs. 15.6%, p = 0.08) was observed with a significant decline in meropenem resistance (34.9% vs. 19.5%, p = 0.03). MDR-bacteria were more frequent in the case of nosocomial infections. Of note, in ascites the majority of the tested bacteria were resistant against ceftriaxone (73.8%) whereas significantly less were resistant against meropenem (27.0%) and vancomycin (25.9%). Conclusions In our tertiary center, distinct ratios of gram-positive infection with overall low ratios of MDR-bacteria were found. Adequate gram-positive coverage in the empiric therapy should be considered. Carbapenem treatment may be omitted even in nosocomial infection. In contrast, 3rd generation cephalosporins cannot be recommended even in community-acquired infection in our cirrhotic population.

Among patients with bronchiectasis, nontuberculous mycobacterial pulmonary disease (NTM-PD) ranged between 1 and 6% and it is suspected that its prevalence is underestimated. Our aim was to evaluate differences in computed tomography (CT) features in patients with bronchiectasis, with and without NTM-PD, in order to facilitate earlier diagnosis in the future. In addition, we evaluated longitudinal changes after successful NTM-PD treatment. One hundred and twenty-eight CTs performed in adults with bronchiectasis were scored for the involvement, type, and lobar distribution of bronchiectasis, bronchial dilatation, and bronchial wall thickening according to Reiff. In addition, associated findings, such as mucus plugging, tree-in-bud, consolidations, ground-glass opacities, interlobular thickening, intralobular lines, cavities, and atelectasis, were registered. Patients with NTM-PD (n = 36), as defined by ATS/IDSA diagnostic criteria, were compared to bronchiectasis patients without NTM-PD (n = 92). In twelve patients with an available consecutive CT scan after microbiological cure of NTM-PD imaging findings were also scored according to Kim and compared in the course. In patients with NTM-PD, there was a higher prevalence of bronchiectasis in the middle lobes (p &lt, 0.001), extended bronchiolitis (p = 0.032) and more small and large nodules (p &lt, 0.001). Furthermore, cavities turned out to be larger (p = 0.038), and walls thickened (p = 0.019) and extended (p = 0.016). Patients without NTM more often showed peripheral ground-glass opacities (0.003) and interstitial changes (p = 0.001). CT findings decreased after successful NTM-PD treatment in the follow-up CT, however, without statistical significance for most features (p = 0.056), but bronchiolitis was the only significantly reduced score item (p = 0.043). CT patterns in patients with bronchiectasis and NTM-PD differ from those of patients with bronchiectasis without NTM-PD, although the findings are non-specific radiological features. Follow-up CT findings after microbiological cure differed interindividual regarding the decline in imaging features. Our findings may help practitioners to identify NTM-PD in patients with bronchiectasis. Further research is needed regarding the use of CT as a potential imaging biomarker for the evaluation of treatment response.

Introduction: Immunodeficiency is a recognised cause of bronchiectasis but individual cohorts are typically too small to describe the demographics and severity of disease in this group. The EMBARC Registry represents a large dataset to address this. Methods: Data submitted from centres to EMBARC from Jan 2015 to June 2019 was studied. Severity of disease was evaluated using the Bronchiectasis severity index (BSI) and quality of life using the QOL-B. Results: Of the 13512 patients included, 491 (3.6%) patients were reported as having bronchiectasis due to immunodeficiency. The most commonly reported cause entered was common variable immunodeficiency (35.8%). Patients with immunodeficiency had a mean age of 56.5 years (sd 17.3), with moderate severity (median BSI 6, IQR 3-10) and symptomatic impairment (QOL-B 63.5, sd 20.6). During follow-up (mean 3 years) patients with immunodeficiency as the reported aetiology did not have a higher exacerbation frequency compared to idiopathic bronchiectasis – rate ratio 0.90 95% CI 0.78-1.04,p=0.15. Interestingly, patients with immunodeficiency had significantly less severe respiratory symptoms using the QOL-B (difference 3.5 points 95% CI 1.61-5.37,p Conclusion: Immunodeficiency accounts for 3.6% of bronchiectasis cases. Patients have a wide spectrum of severity but on average less severe symptoms compared to patients with other aetiologies.

Introduction: Alpha-1 antitrypsin deficiency is a recognised cause of bronchiectasis. There are few large cohorts, however, describing the frequency, clinical characteristics and severity of disease in this population. Methods: The European Bronchiectasis Registry (EMBARC) was studied from Jan 2015 to June 2019. Severity of disease was evaluated using the Bronchiectasis severity index (BSI) and quality of life using the QOL-B questionnaire. Results: Of 13512 patients included in the analysis, 3390 patients were tested for alpha-1 antitrypsin deficiency. Rates of testing by country varied from 0% in several countries to 67.5% in Portugal (UK 12.8%, Germany 40.2%, Italy 28.3%, Spain 43.8%) . Alpha-1 deficiency was identified in 77 patients (2.3%). Mean age was 61 years (SD 12), 47 females (61.0%). The mean bronchiectasis severity index was 7.8 points (SD 5.2). 26, 20 and 31 patients were classified as mild, moderate and severe respectively. The median exacerbation rate was 2 per year (range 0-13). Mean FEV1 was 70.2% (SD 32). Patients had moderate symptomatic impairment (mean QOL-B symptom score 58.7 SD 23). The most common organisms were Pseudomonas aeruginosa (23.4%), Enterobacteriales (9.1%), Staphylococcus aureus (9.1%), Haemophilus influenzae (6.5%) and NTM (5.2%). During follow-up (mean 3 years) patients with alpha-1 antitrypsin deficiency had a similar rate of exacerbations as patients with idiopathic bronchiectasis- rate ratio 1.12 95% CI 0.80-1.58,p=0.51. Conclusions: Alpha-1 antitrypsin deficiency is an uncommon cause of bronchiectasis in the EMBARC registry, but estimates are limited by infrequent testing. A study to determine the true prevalence of alpha-1 antitrypsin deficiency through systematic testing is needed.

Background: Randomized Trials in bronchiectasis may have been underpowered due to unexpectedly large “placebo effects”. We used a combination of meta-analysis and analysis of the European Bronchiectasis Registry (EMBARC) to quantify “placebo effects” in randomized trials in bronchiectasis Method: We searched electronic databases from 1990-2019, to identify double blind, randomized, placebo-controlled trials of drug treatment in bronchiectasis. We defined the “placebo effects” by comparing the outcomes at the end of placebo treatment and at baseline prior to treatment. EMBARC registry data were employed to model changes in exacerbation rate and quality of life (QoL) Result: 27 trials were included for meta-analysis (N=1739 placebo-treated patients). Placebo treatment was associated with significantly reduced exacerbation rate (mean difference [MD] -1.79, -2.03 to -1.56; p Conclusion: Placebo treatment was associated with improved QoL and reduced exacerbation in bronchiectasis. Enrolling patients with 1 or more exacerbation may be an optimal inclusion criteria

Bronchiectasis is a chronic disease with a variety of aetiologies that influence the clinical presentation and management of the disease. One of these aetiologies is allergic bronchopulmonary aspergillosis (ABPA). Methods: 27 of 1000 patients (2.7%) included in PROGNOSIS had ABPA as aetiology of bronchiectasis (per investigator judgment). ABPA testing was the most common aetiological test performed in 58% of subjects. Comparison was made to 973 adults with bronchiectasis caused by any other aetiology. Results: Mean age was 63 years in ABPA compared to 61 years (p=0.410), 37% were male (vs 41%; p=0.694). Median annual exacerbation rate was 2 (each; p=0.47). Although mean ppFEV1 differed from 59.7% (95% CI: 52.5-77.0) to 70.6% (95% CI: 66.9-71.3), there was no significant difference (p=0.842). Radiological features showed predominant involvement of upper lobes (left 70.4% vs. 35.5%, p Method: Adults with ABPA as aetiology of bronchiectasis show mainly upper lobe involvement and cystic bronchiectasis with no differences in frequency of positive fungal cultures. The majority is treated with OCS, only two-thirds received ICS, antimycotics were infrequently used. Further research is warranted, especially as ABPA is considered a treatable trait in bronchiectasis.

Background: Primary ciliary dyskinesia (PCD) is a rare genetic disorder which requires a complex diagnostic workup. Thus, an easy and widely available screening method would be helpful to identify patients who need a further diagnostic workup for PCD. Objectives: The aim of the study was to develop and validate a computed tomography (CT) score for PCD to facilitate etiological diagnosis in adults with bronchiectasis. Method: Chest CTs from 121 adults with bronchiectasis were scored for bronchiectasis morphology, distribution, and associated findings. Patients with and without the etiological diagnosis of PCD (46 and 75, respectively) were compared. Significantly, different imaging findings (p < 0.05) in univariate analysis were considered for multivariate analysis. Distinct findings were used to build the score. Based on this score, receiver operating characteristic (ROC) curve analysis was performed. The score was validated with 2 independent cohorts, another cohort from the same institution with 56 patients (28 with PCD) and an external cohort from another referral center with 172 patients (86 with PCD). Results: The following parameters predicted PCD in adults with bronchiectasis and were included in the score with weighting according to their regression coefficients: 2 points were given for predominance in the middle/lower lobe, 2 points for tree-in-bud pattern, 2 points for atelectasis or prior resection of a middle/lower lobe, and 3 points for absence of emphysema and fibrosis. Situs inversus was only observed in subjects with PCD (Kartagener syndrome) and, thus, was not used in the primary ciliary dyskinesia computed tomography (PCD-CT) score as group comparisons could not be performed. ROC curve analysis revealed an area under the curve (AUC) of 0.90 (95% CI 0.85–0.96). Youden index was the highest at a threshold of >6 with a sensitivity of 83% and a specificity of 83%. In the validation cohorts, ROC curve analysis confirmed the performance of the score with an AUC of 0.83 (95% CI 0.72–0.94) in the first validation cohort and 0.79 (95% CI 0.73–0.86) in the external validation cohort. Conclusions: The PCD-CT score provides the first validated CT score for PCD and helps physicians in identifying adult bronchiectasis patients who require further diagnostic workup. Key message: The PCD-CT score provides the first validated CT score to assist physicians in identifying adult bronchiectasis patients who require a further diagnostic workup for PCD. It potentially improves earlier recognition of this rare and underdiagnosed disease.

Background: YNS is an orphan disease, the prevalence is estimated to be Objective: To describe the phenotype of patients with YNS and bronchiectasis to identify targets for future research regarding the pathogenesis, diagnosis and specific treatment. Methods: Data from the German and European bronchiectasis registries PROGNOSIS and EMBARC was analyzed descriptively. Differences between subjects with YNS (pooled data from PROGNOSIS and EMBARC) and other etiologies of bronchiectasis (data from PROGNOSIS) were assessed by Fisher´s exact, Chi2 and Mann-Whitney-U test. Results: Overall, 31 patients in PROGNOSIS and EMBARC had YNS as aetiology. Median age was 67 yrs; 58% were female, the median (IQR) FEV1% predicted was 68% (59-83) and 32% grew Pseudomonas aeruginosa in sputum cultures. Bronchiectasis were seen predominantly in lower lobes. Compared to 994 subjects without YNS from PROGNOSIS registry significant differences were observed with regard to the proportion with chronic rhinosinusitis (CRS) (52% in YNS vs. 30%; p=0.016) and the median [IQR] exacerbation frequency (2 [1-6] vs. 1 [0-3]; p=0.009). Conclusion: Our data suggest that YNS is a rare cause of bronchiectasis, which may be associated with frequent exacerbations and rhinosinusitis. Further multicenter research is warranted.

Purpose: Aim of this multicenter study was to evaluate specific features of Primary Ciliary Dyskinesia (PCD) in computed tomography and to develop and validate a CT score for PCD to facilitate diagnosis. Methods: 121 CT in adult patients with bronchiectasis were scored. In univariate analysis imaging findings with p Results: In multivariate analysis, Situs inversus (p Conclusions: PCD-CT provides the first validated CT score for PCD to help practitioners in identifying bronchiectasis patients who require a further diagnostic workup for PCD.

Background: The relevance of nontuberculous mycobacteria (NTM) among subjects with bronchiectasis in Germany is unknown. In this regard, the German Bronchiectasis Registry PROGNOSIS is an excellent opportunity to provide further insights. Methods: We analyzed the first consecutively recruited and validated 1000 baseline datasets of PROGNOSIS and compared subjects with a history of NTM and/or a positive respiratory culture at baseline (NTM group) to subjects without such history and no isolation (non-NTM group). Differences between groups were assessed by univariate analysis. Results: Overall, only 56% received AFB cultures at baseline. Among 49 subjects with a positive culture MAC (76%), M. kansasii (14%) and M. gordonae (12%) were the most frequent NTM species. Using the above definition we identified 73 NTM patients (7.3%). Those were older (mean age 63 vs. 59 years; P=0.041), more often female (69 vs. 59%, P=0.11) and had a lower median BMI (23.1 vs. 24.2 kg/m²; P=0.046). Upper airway involvement, PCD as an etiology and ICS use was less frequent (18 vs. 34%, P=0.004; 3 vs. 9%, P=0.055; and 3 vs. 19%, P Conclusion: Patients with bronchiectasis and NTM exhibit distinct clinical features. Comparing our findings to those of the US Bronchiectasis Research Registry we found similarities and differences, which warrant further investigation by multivariate analysis.

Patients with clinically significant bronchiectasis featuring an eosinophilic inflammatory endotype who were treated with add-on mepolizumab or benralizumab showed a significant improvement of FEV1, symptom burden and quality of lifehttp://bit.ly/2AZvBLm

This study sought to investigate the prognostic importance of a low diffusion capacity of the lung for carbon monoxide (DLCO) in patients with a catheter-based diagnosis of pulmonary hypertension due to heart failure with preserved ejection fraction (PH-HFpEF).In patients with pulmonary arterial hypertension, a low DLCO is associated with poor outcome. It is unclear whether the same is true in patients with PH-HFpEF.This study retrospectively analyzed clinical characteristics, smoking history, lung function measurements, chest computed tomography, hemodynamics, and survival in 108 patients with PH-HFpEF. The presence of post-capillary PH was determined by right heart catheterization. Patients with moderate or severe lung function abnormalities were excluded.On the basis of previous studies and receiver-operating characteristic curve analysis, the study cohort was divided into patients with a DLCO 45% of the predicted value (DLCO45%, low DLCO; n = 52) and patients with a DLCO ≥45% of the predicted value (DLCO≥45%; n = 56). DLCO45% was associated with male sex (odds ratio [OR]: 2.71; 95% confidence interval [CI]: 1.05 to 6.99; p = 0.039) and smoking history (OR: 5.01; 95% CI: 1.91 to 13.10; p 0.001). There were no correlations between DLCO and other lung function parameters and hemodynamics. Compared with patients with DLCO≥45%, patients with DLCO45% had a significantly worse outcome (survival rate at 3 years 36.5% vs. 87.8%, p 0.001 by log-rank analysis). Cox proportional hazard analysis identified DLCO45% as an independent predictor of death (hazard ratio: 6.6; 95% CI: 2.6 to 16.9; p 0.001).In patients with PH-HFpEF, a low DLCO is strongly associated with mortality.

Background Lung transplantation (LTx) is a well-established treatment for end-stage pulmonary disease. However, data regarding microbiology and outcome of patients with non-cystic fibrosis bronchiectasis (NCFB) after lung transplantation are limited. Methods A retrospective analysis between August 1992 and September 2014 of all patients undergoing lung transplantation at our program of all recipients with a primary diagnosis of bronchiectasis was performed. Microbiology of sputum and bronchoalveolar lavage specimens, lung function and clinical parameters pre- and post-LTx were assessed retrospectively. Overall survival was compared to the total cohort of lung transplant recipients at institution. The survival and development of chronic lung allograft dysfunction (CLAD) was compared in patients with and without chronic Pseudomonas aeruginosa (PSA) infection after LTx. Results 34 patients were transplanted. Median age at transplantation was 40 (IQR 33–52) years. The most common etiologies of bronchiectasis were idiopathic (41%), chronic obstructive pulmonary disease (COPD) (21%) and post-infectious (15%). The most common organism of pre- and posttransplant chronic airway infection was PSA. One-year Kaplan-Meier survival for patients with bronchiectasis was 85% and 5-year survival was 73% and similar to the entire cohort. All three patients with an associated diagnosis of immunodeficiency died due to infection and sepsis within the first year. Patients with persistent colonization with Pseudomonas aeruginosa after transplantation had worse long-term survival by trend and developed chronic lung allograft dysfunction more frequently. Conclusions Overall survival of patients with bronchiectasis after LTx is comparable to other underlying diseases. A reduced survival was observed in patients with the underlying diagnosis of immunodeficiency.

We analyzed routine statutory health insurance claim data to determine prevalence of nontuberculous mycobacterial pulmonary disease in Germany. Documented prevalence rates of this nonnotifiable disease increased from 2.3 to 3.3 cases/100,000 population from 2009 to 2014. Prevalence showed a strong association with advanced age and chronic obstructive pulmonary disease.

Background Incidence and prevalence of patients with non-cystic fibrosis bronchiectasis (NCFB) appear to be increasing worldwide but supporting epidemiological data are scarce. This study assesses the incidence of NCFB patients in Germany in 2013 and analyzes comorbidities and basic patterns of resource use. Methods A representative sample of 3.988.648 anonymized persons covered by German public statutory health insurances was used to identify incident patients with NCFB in 2013. Results After extrapolation to the general population of the 728 patients found in the reference insurance database, we estimate that a total of 17,095 NCFB patients were newly diagnosed across the country in 2013 as having NCFB. This corresponds to an incidence of 21.23 per 100.000 inhabitants. The majority of NCFB patients (98.4%) was at least 18 years old, and 52.7% of the NCFB patients were male. Trend analysis shows a rise of NCFB incidence in Germany from 2011 through 2013. COPD (41.4%), asthma (32.8%) and gastroesophageal reflux (18.3%) were the most frequent predisposing conditions. Coronary heart disease was observed in more than one quarter of NCFB patients (28.2%). 58.4% of the NCFB outpatients received antiobstructive inhalative medication. Of the adult NCFB patients, 51.6% were prescribed antibiotics to treat NCFB by settled doctors (outpatient treatment); 51.5% of those patients were males. The peak of antibiotic treatment was observed in the 75–79 age group for males and 70–74 and 75–79 years for females. The majority of diagnosed patients (54.1%) received at least two prescriptions during 2013. Bacterial pathogens were coded for a total of 10.7% of NCFB patients, while Pseudomonas aeruginosa was only documented in 2.3%. Among those diagnosed in 2013, 8.0% of the adult NCFB patients who received antibiotic treatment had to be hospitalized. Conclusions Although hospital admissions due to exacerbation in the first year of diagnosing NCFB are not rare, outpatient burden and costs must also be considered a major part of care. Given the increasing recognition of NCFB, a better understanding of the economic burden of the disease is required, with a view towards improving patient management. For this, more detailed, prospective studies are needed.

Bronchiectasis is a chronic and progressive disease, which frequently is associated with a significant symptom burden and which may require intensive treatment. To date there is no data how many patients with bronchiectasis suffer from eosinophilia and how monoclonal anti-allergic antibodies work in these patients. The aim of this case series study was to describe the effect of mepolizumab in patients with bronchiectasis and underlying eosinophilic conditions. 300 patients are in regular follow up in the adult bronchiectasis clinic of the Hannover Medical School and 7% of patients had an eosinophilic blood count of more than 300 per cubic millimeter. In 15 patients of this cohort we decided to treat the disease with the Anti- IL5 antibody mepolizumab due to high exacerbation rate, low quality of life and high corticosteroid use. Overall, twelve patients of the cohort receiving mepolizumab had follow up data after 3 and 6 months of therapy. After six months of therapy the median (IQR) eosinophilic cell count decreased significantly from 1000 (500-1900) to 100 (0-200) per cubic millimeter (p=0.001). The median (IQR) annual rate of exacerbations improved with 1 (0-3) per year after 6 months of therapy, as compared with 3 (1-5) per year at baseline. Similar results showed the median (IQR) mMRC dyspnoe scale, which decreased from 2 (1-3) to 0 (0-1) (p=0.004). In the patient-reported outcomes the median (IQR) quality of life measured in a visual analog scale (1-10) improved significantly from 4 (3-6) to 6.5 (5-7) (p=0.01). In conclusion, patients with bronchiectasis and eosinophilia had significantly less dyspnea; a higher quality of life score and lower eosinophilic cell count under the therapy of mepolizumab.

We investigated associations between the Quality of Life Questionnaire-Bronchiectasis (QoL-B) scales and symptom burden as well as markers of disease severity within the German Bronchiectasis Registry PROGNOSIS. The German translation of the QoL-B V.3.0, which uses 8 scales (Respiratory Symptoms [RS], Physical Functioning [PF], Vitality [V], Role Functioning [RF], Health Perceptions [HP], Emotional Functioning [EF], Social Functioning [SF] and Treatment Burden [TB]), is routinely applied at baseline. Between Jun 2015 and Feb 2018, 1043 subjects were recruited from 38 centers (median age 61 years, 59% female). At baseline, up to 95% of subjects completed the QoL-B. All scales were significantly associated with higher MRC dyspnea scores (p Our analysis confirms that the QoL-B is a feasible tool to determine quality of life in this representative cohort of German patients with bronchiectasis.

Bronchiectasis represents the final pathway of several infectious, genetic, immunologic or allergic disorders. Accurate and prompt identification of the underlying cause is a key recommendation of several international guidelines, in order to tailor treatment appropriately. Primary ciliary dyskinesia (PCD) is a genetic cause of bronchiectasis in which failure of motile cilia leads to poor mucociliary clearance. Due to poor ciliary function in other organs, individuals can suffer from chronic rhinosinusitis, otitis media and infertility. This paper explores the current literature describing why, when and how to investigate PCD in adult patients with bronchiectasis. We describe the main PCD diagnostic tests and compare the two international PCD diagnostic guidelines. The expensive multi-test diagnostic approach requiring a high level of expertise and specialist equipment, make the multifaceted PCD diagnostic pathway complex. Therefore, the risk of late or missed diagnosis is high and has clinical and research implications. Defining the number of patients with bronchiectasis due to PCD is complex. To date, few studies outlining the aetiology of adult patients with bronchiectasis conduct screening tests for PCD, but they do differ in their diagnostic approach. Comparison of these studies reveals an estimated PCD prevalence of 1–13% in adults with bronchiectasis and describe patients as younger than their counterparts with moderate impairment of lung function and higher rates of chronic infection with Pseudomonas aeruginosa. Diagnosing PCD has clinical, socioeconomic and psychological implications, which affect patients’ life, including the possibility to have a specific and multidisciplinary team approach in a PCD referral centre, as well as a genetic and fertility counselling and special legal aspects in some countries. To date no specific treatments for PCD have been approved, standardized diagnostic protocols for PCD and recent diagnostic guidelines will be helpful to accurately define a population on which planning RCT studies to evaluate efficacy, safety and accuracy of PCD specific treatments.