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Background The survival of women with cystic fibrosis (CF) is lower than that of men by approximately 5 years. While various factors have been put forward to account for this discrepancy, no specific reasons have been established. Our hypothesis was that anatomical-structural involvement is more pronounced in women with CF than in men and that this is reflected in thoracic HRCT findings. Material and methods We performed a prospective multicentre study, in which adult patients were consecutively included over 18 months. Chest HRCT was performed, and findings were scored by 2 thoracic radiologists using the modified Bhalla system. We also studied respiratory function, applied the CFQR 14+ questionnaire, and collected clinical variables. Results Of the 360 patients followed up in the participating units, 160 were eventually included. Mean age was 28 years, and 47.5% were women. The mean ± SD global score on the modified Bhalla score was 13.7 ± 3.8 in women and 15.2 ± 3.8 in men (p = 0.024). The highest scores were observed for sacculations, bronchial generations, and air trapping in women. Women had lower BMI, %FEV1, %FVC, and %DLCO. Similarly, the results for the respiratory domain in CFQR 14+ were worse in women, who also had more annual exacerbations. Conclusions This is the first study to provide evidence of the implication of sex differences in HRCT findings in patients with CF. Women with CF present a more severe form of the disease that results in more frequent exacerbations, poorer functional and nutritional outcomes, deterioration of quality of life, and greater structural damage.

The objective of this study was to analyze the predictive value of the modified Bhalla score in high-resolution computed tomography (HRCT) for assessment of pulmonary exacerbations (PEx) in cystic fibrosis (CF) patients. We also describe the relationship between this score and pulmonary function test results. We performed a multicenter and prospective study where adult patients with CF were included consecutively over 18 months. All patients underwent HRCT with acquisition in inspiration and expiration. The results were analyzed by an expert radiologist who assigned a modified Bhalla score value. Lung function was also assessed, and clinical variables were collected. Follow-up lasted approximately 1 year, and PEx were registered. The study population comprised 160 subjects selected from 360 CF patients monitored in the participating CF units. The mean age was 28 years, 47.5% were women, and mean forced expiratory volume in 1 s (FEV1) was 67.5%. The mean global modified Bhalla score was 14.5 ± 0.31 points. Pulmonary function test (PFT) results and the modified Bhalla score correlated well, mainly forced vital capacity (FVC) and FEV1. We constructed a statistical model based on the overall Bhalla score to predict the number of PEx. The overall modified Bhalla score can predict future PEx in CF patients. This useful tool can help to prevent PEx in higher risk patients. • Pulmonary function test results and the modified Bhalla score correlated well with FVC and FEV1. • The total modified Bhalla score can predict the number of exacerbations in adult CF patients. • Our findings highlight the need to establish a unified protocol for chest HRCT during the follow-up of adult patients with CF in order to anticipate possible complications and determine their impact on pulmonary function.

Background Availability of clinically effective and cost-effective treatments for severe asthma would be beneficial to patients and national healthcare systems. The aim of this study was to evaluate clinical outcomes and healthcare expenditure after incorporating benralizumab into the standard treatment of refractory eosinophilic asthma. Methods This was a cross-sectional multicentre study of consecutive patients with refractory eosinophilic asthma who received treatment with benralizumab during at least 12 months. Patient follow-up was performed in specialised severe asthma units. The main effectiveness parameters measured were: the avoidance of one asthma exacerbation, a 3-point increase in the asthma control test (ACT) score, and the difference in utility scores (health-related quality of life) between a 1-year baseline treatment and 1-year benralizumab treatment. The health economic evaluation included direct costs and incremental cost-effectiveness ratios (ICERs). Results After 1 year of treatment with benralizumab, patients with refractory eosinophilic asthma showed an improvement in all the effectiveness parameters analysed: improvement of asthma control and lung function, and decrease in the number of exacerbations, oral corticosteroid (both as corticosteroid courses and maintenance therapy), and inhaled corticosteroid use. The total annual cost per patient for the baseline and benralizumab treatment periods were €11,544 and €14,043, respectively, reflecting an increase in costs due to the price of the biological agent but a decrease in costs for the remaining parameters. The ICER was €602 per avoided exacerbation and €983.86 for every 3-point increase in the ACT score. Conclusions All the pharmacoeconomic parameters analysed show that treatment with benralizumab is a cost-effective option as an add-on therapy in patients with refractory eosinophilic asthma.

Background: Nebulised antibiotics are habitually used in patients with bronchiectasis, but the use of dry powder inhaled antibiotics (DPIA) in these patients is extremely limited. This study seeks to analyse the efficacy and safety of DPIA in bronchiectasis patients. Material and methods: Multi-centre study of historic cohorts. All the hospital centres in Spain were contacted in order to collect data on patients with a diagnosis of bronchiectasis who had taken at least one dose of DPIA. Its efficacy was analysed in clinical, functional and microbiological terms by comparing the year before and the year after the prescription of DPIA. Adverse effects and variables associated with these effects, or any need to withdraw the drug, were also analysed. Results: 164 patients from 33 Spanish centres were included, 86% and 14% of these were treated with dry powder colistin and tobramycin, respectively. Chronic bronchial infection by Pseudomonas aeruginosa was present in 86% of these patients, and DPIA significantly reduced the number of exacerbations, the quantity and purulence of sputum and the isolation of pathogenic microorganisms. The most common adverse effect was cough (40%), particularly in cases of Chronic Obstructive Pulmonary Disease (COPD) and a previous cough and in those patients who had difficulties in handling the device. These factors were associated with a higher level of withdrawal of the treatment. There were no serious adverse effects. Conclusions: Our study suggests that DPIA are clinically efficacious and safe for treating bronchiectasis patients. Cough was shown to be the most common side-effect and reason for withdrawal of the treatment.

BackgroundBenralizumab is a monoclonal antibody that binds to the human interleukin-5 (IL-5) receptor (IL-5R), thereby preventing IL-5 from binding to its receptor and inhibiting differentiation and maturation of eosinophils in the bone marrow. Because of its recent marketing approval, sufficient real-life evidence is lacking to confirm the efficacy and safety data from clinical trials. The purpose of this study was to evaluate the efficacy and safety of benralizumab for the treatment of severe refractory eosinophilic asthma in a real-world cohort of patients.MethodsThis was a cross-sectional multicentre study of consecutive patients with severe refractory eosinophilic asthma who received treatment with benralizumab during at least 6 months. Patient follow-up was performed in specialised severe asthma units.ResultsA total of 42 patients were enrolled and treated with benralizumab. Asthma control, as measured by the asthma control test (ACT), improved in all patients both at 3 months of treatment compared with baseline (13.9 ± 4 vs 20.1 ± 3.7,p p = 0.037). Similarly, the number of emergency department visits decreased both at 3 months compared with baseline (1 [IR:0.7] vs 0 [IR:0.75],p p = 0.012). Reductions in the number of oral corticosteroid cycles, percentage of corticosteroid-dependent patients, and mean daily dose of oral or inhaled corticosteroid were also evidenced. Finally, mean lung function improvement was 291 mL (p p p = 0.002). Side effects were mild and did not lead to treatment discontinuation.ConclusionsThis study confirms the efficacy and safety of benralizumab in a real-life setting with improved asthma control and lung function, and a reduced oral and inhaled corticosteroid use as well as fewer emergency department visits. In addition to a rapid initial improvement, it appears that patients continue to improve during the first 6 months of treatment.

Background Omalizumab is a fully humanized monoclonal antibody indicated as add-on therapy to improve asthma control in patients with severe persistent allergic asthma. Aims The aim of this study was to evaluate social, healthcare expenditure and clinical outcomes changes after incorporating omalizumab into standard treatment in the control of severe asthma. Methods In this multicentre retrospective study, a total of 220 patients were included from 15 respiratory medicine departments in the regions of Andalusia and Extremadura (Spain). Effectiveness was calculated as a 3-point increase in the Asthma Control Test (ACT) and a reduction in the annual number of exacerbations. The economic evaluation included both direct and indirect costs. Incremental cost-effectiveness ratio (ICER) was calculated. Results from the year before and the year after incorporation of omalizumab were compared. Results After adding omalizumab, improvement of lung function, asthma and rhinitis according to patient perception, as well as the number of exacerbations and asthma control measured by the ACT score were observed. Globally, both healthcare resources and pharmacological costs decreased after omalizumab treatment, excluding omalizumab cost. When only direct costs were considered, the ICER was €1712 (95% CI 1487–1995) per avoided exacerbation and €3859 (95% CI 3327–4418) for every 3-point increase in the ACT score. When both direct and indirect costs were considered, the ICER was €1607 (95% CI 1385–1885) for every avoided exacerbation and €3555 (95% CI 3012–4125) for every 3-point increase. Conclusions Omalizumab was shown to be an effective add-on therapy for patients with persistent severe asthma and allowed reducing key drivers of asthma-related costs.

Introduction Sexual limitations play an important role in the quality of life of patients with chronic diseases. Very limited information is available on the impact of asthma on the sexual functioning of these individuals. Materials and methods Cross-sectional, observational, multicenter study. Asthma patients and healthy individuals were recruited. All subjects participated in an interview in which demographic and clinical data were recorded, and completed the Goldberg Anxiety-Depression Scale (GADS) to evaluate the presence of concomitant psychiatric disease. Men also completed the International Index of Erectile Function (IIEF), and women, the Female Sexual Function Index (FSFI). Results A total of 276 cases were included, comprising 172 asthma patients (63 men and 109 women) with a mean age of 42 (±14) years, and 104 controls (52 men and 51 women) with a mean age of 39 (±12) years. Time since onset of asthma was 15 years and severity distribution was: 6.4% intermittent, 17.9% mild persistent, 47.4% moderate, and 28.2% severe. Disease was considered controlled in 57.7%, partially controlled in 28.2%, and uncontrolled in 14.1%. Women with asthma had greater sexual limitations than women in the control group, with a total FSFI score of 22.1 (±9) compared to 26.5 (±6.8), respectively (P Conclusions Asthma is associated with a poorer sexual quality of life among patients. These results should arouse the interest of healthcare professionals in detecting and alleviating possible sexual limitations among their asthma patients in routine clinical practice.

Resumen Introduccion Las limitaciones de la vida sexual de los pacientes con enfermedades cronicas desempenan un importante papel en su calidad de vida. La informacion disponible sobre el impacto de la enfermedad asmatica en la vida sexual de las personas es muy limitada. Material y metodos Estudio transversal, observacional y multicentrico. Se reclutaron pacientes asmaticos y sujetos sanos, a los que se les efectuo una entrevista en la que se recogieron datos demograficos y clinicos y en donde ademas cumplimentaron los siguientes cuestionarios: la escala Goldberg Anxiety -Depression Scale (GADS) para la valoracion de la existencia de comorbilidad psiquiatrica, y en varones el Indice Internacional de la Funcion Erectil (IIEF) y en mujeres el Indice de la Funcion Sexual Femenina (FSFI). Resultados Se incluyeron un total de 276 casos, de los que 172 eran asmaticos (63 hombres y 109 mujeres) con una media de edad de 42 (± 14) anos y 104 controles (53 hombres y 51 mujeres) con una media de edad de 39 (± 12) anos. El asma presentaba una evolucion de 15 anos y su gravedad se distribuia: 6,4% intermitente, 17,9% persistente leve, 47,4% moderada y 28,2% grave. La enfermedad se considero controlada en un 57,7%, parcialmente controlada en un 28,2% y no controlada en un 14,1%. Las mujeres asmaticas presentaron una mayor limitacion de la vida sexual que las mujeres del grupo control, con una puntuacion total del FSFI de 22,1 (± 9) frente a 26,5 (± 6,8), respectivamente (p Conclusiones La enfermedad asmatica condiciona una peor calidad de vida sexual en las personas afectadas. Estos resultados deberian promover, en la practica clinica habitual, el interes de los profesionales sanitarios por determinar y paliar las posibles limitaciones sexuales de sus pacientes con asma.

Background: Severe asthma is a serious condition with a significant burden on patients' morbidity, mortality, and quality of life. Some biological therapies targeting the IgE and interleukin-5 (IL5) mediated pathways are now available. Due to the lack of direct comparison studies, the choice of which medication to use varies. We aimed to explore the beliefs and practices in the use of biological therapies in severe asthma, hypothesizing that differences will occur depending on the prescribers’ specialty and experience. Methods: We conducted an online survey composed of 35 questions in English. The survey was circulated via the INterasma Scientific Network (INESNET) platform as well as through social media. Responses from allergists and pulmonologists, both those with experience of prescribing omalizumab with (OMA/IL5) and without (OMA) experience with anti-IL5 drugs, were compared. Results: Two hundred eighty-five (285) valid questionnaires from 37 countries were analyzed. Seventy-on percent (71%) of respondents prescribed biologics instead of oral glucocorticoids and believed that their side effects are inferior to those of Prednisone 5 mg daily. Agreement with ATS/ERS guidelines for identifying severe asthma patients was less than 50%. Specifically, significant differences were found comparing responses between allergists and pulmonologists (Chi-square test, p < 0.05) and between OMA/IL5 and OMA groups (p < 0.05). Conclusions: Uncertainties and inconsistencies regarding the use of biological medications have been shown. The accuracy of prescribers to correctly identify asthma severity, according to guidelines criteria, is quite poor. Although a substantial majority of prescribers believe that biological drugs are safer than low dose long-term treatment with oral steroids, and that they must be used instead of oral steroids, every effort should be made to further increase awareness. Efficacy as disease modifiers, biomarkers for selecting responsive patients, timing for outcomes evaluation, and checks need to be addressed by further research. Practices and beliefs regarding the use of asthma biologics differ between the prescriber's specialty and experience; however, the latter seems more significant in determining beliefs and behavior. Tailored educational measures are needed to ensure research results are better integrated in daily practice.

Approximately 5%-10% of asthmatics suffer from severe asthma. New biological treatments represent a great opportunity to reduce asthma burden and to improve asthma patients' lives. Reslizumab will soon be available in several European countries. This anti-IL-5 IgG4/kappa monoclonal antibody, administered intravenously at a dose of 3 mg/kg over 20-50 minutes every 4 weeks, has been shown to be safe and effective in patients with 400 eosinophils/mu L or more in their peripheral blood. The clinical effects in reducing asthma exacerbations and in improving the quality of life and lung function are clear, but further research is needed to determine the best biological compound for a specific cluster of patients. Research data have shown that in patients who were expressing other clinical features of eosinophilic inflammation over asthma (rhinosinusitis and nasal polyposis), the clinical benefit of reslizumab was greater. Furthermore, it has also been observed that in patients with unsatisfactory response to mepolizumab, reslizumab is able to significantly improve the clinical and biological parameters. The aim of personalized medicine is to provide the right drug to the right patient at the right dose at the right moment. The biological treatments that were developed to modify specific pathological pathways not only provide us with the tools for the management of asthma patients but also clarify the biological mechanisms involved in its pathogenesis.

Background: Some studies have reported a high prevalence of bronchiectasis in patients with uncontrolled asthma, but the factors associated with this condition are unknown. The objective of this study was to determine the prevalence of bronchiectasis in uncontrolled moderate-to-severe asthma and to identify risk factors and their correlation with bronchiectasis in these patients. Methods: This is a prospective study of data from consecutive patients with uncontrolled moderate-to-severe asthma. Diagnosis of bronchiectasis was based on high-resolution computed tomography. A prognostic score was developed using a logistic regression model, which was used to determine the factors associated with bronchiectasis. Results: A total of 398 patients (60% with severe asthma) were included. The prevalence of bronchiectasis was 28. 4%. The presence of bronchiectasis was associated with a higher frequency of chronic expectoration (OR, 2.95; 95% CI, 1.49-5.84; p = 0.002), greater severity of asthma (OR, 2.43; 95% CI, 1.29-4.57; p = 0.006), at least one previous episode of pneumonia (OR, 2.42; 95% CI, 1.03-5.69; p = 0.044), and lower levels of FeNO (OR, 0.98; 95% CI, 0.97-0.99; p = 0.016). The NOPES score was developed on the basis of these variables (FeNO[cut off point 20.5 ppb], Pneumonia, Expectoration and asthma Severity), and it ranges from 0 to 4 points, where 0 means "no risk" and 4 corresponds to "high risk". The NOPES score yielded an AUC-ROC of 70% for the diagnosis of bronchiectasis, with a specificity of 95%. Conclusions: Almost a third of the patients with uncontrolled moderate-to-severe asthma had bronchiectasis. Bronchiectasis was related to the severity of asthma, the presence of chronic expectoration, a previous history of pneumonia, and lower levels of FeNO. The NOPES score is an easy-to-use scoring system with a high prognostic value for bronchiectasis in patients with uncontrolled moderate-to-severe asthma.

Objectives: Comparison of adherence to nebulized sodium colistimethate (Promixin) and agreement between the multidimensional FACED, E-FACED and BSI scales in a cohort of patients with non-cystic fibrosis BQ and chronic bronchial infection (CBI) by PsA. Patients and Methods: A multicenter, observational, longitudinal prospective study of a cohort of adults with non-CF BQ, all with PsA CBI and treated with Promixin. FACED, EFACED and BSI scales in the previous year, their modification after a year of treatment with the nebulized therapy and the concordance between them are collected. Results: 99 patients were started average age 63±14.7 years. In the year prior to treatment FEV1 was 55±19%, exacerbations 3.8±2.6 and 0.5±0.7 hospital admissions/year. At the end FEV1 was 59±22%, exacerbations/year 1.4±1.2 and hospital admissions/year 0.26±0.6. In the pretreatment assessment there is a low concordance between the EFACED and BSI scales (R=0,248; p Conclusions: In our cohort there is good adherence to nebulized treatment with Promixin® and good nebulizer management, regardless of the severity of BQ. These results have their clinical impact in terms of functional improvement, reduction of exacerbations and changes in the level of severity prior to treatment. Given the concordance between the prognostic scales, both could be applicable to assess sensitivity to change in a therapeutic intervention with nebulized antibiotics.

Objective: To assess the clinical and economic impact, in terms of reduction of costs associated with exacerbations, in a cohort of patients with non-cystic fibrosis (non-CF) BQ and PsA CBI treated with Promixin. Patients and Methods: A multicenter, observational, longitudinal prospective study of a cohort of adults with non-CF BQ and PsA CBI treated with Promixin. Data were collected in the year before and after nebulized treatment (pulmonary function, exacerbations without hospital admission, mean per pat/y and total number of admissions and per pat/y). Results: 86 pat, 45 males, 62±15 y. Results are shown in the following data table. Conclusions: Adherence to nebulized treatment is excellent in our patients. In clinical terms, this situation has allowed an improvement in lung function and a significant reduction in the number and severity of exacerbations and hospital admissions. Regarding this issue, the average cost per patient and year of treatment has been reduced by more than €1500 after onset of nebulized therapy.

Objetivo La desnutricion es frecuente en pacientes con fibrosis quistica (FQ) e implica un desequilibrio entre la ingesta y los requerimientos. Nuestro objetivo ha sido calcular el gasto energetico en reposo (GER) mediante calorimetria indirecta en adultos con FQ, compararlo con las formulas estimativas habitualmente empleadas y valorar la influencia de parametros clinicos sobre el GER. Pacientes y metodos Estudiamos a 21 pacientes con FQ que se encontraban estables clinicamente. Se efectuaron medidas de parametros antropometricos, dinamometria de mano, impedanciometria bioelectrica y del GER medido por calorimetria indirecta (CI) . Estudiamos la concordancia entre los valores del GER medidos y estimados por las formulas de la Organizacion Mundial de la Salud (OMS) y de Harris- Benedict (HB) mediante el coeficiente de correlacion intraclase y el metodo de Bland-Altman. Resultados Las ecuaciones infraestimaron el GER en mas del 90% de los casos. La concordancia entre la CI y la estimada por las formulas fue escasa (para OMS, 0,47, y para HB, 0,41). Mediante el metodo de Bland-Altman observamos un sesgo variable entre la CI y las formulas, independiente de los valores del GER. La diferencia entre la CI respecto de la estimada por la formula de la OMS fue significativamente mayor en homocigoticos ΔF508 y en los pacientes con insuficiencia pancreatica exocrina frente al resto. Conclusiones En adultos con FQ, las formulas de la OMS y de HB infraestiman el GER. Hay una baja concordancia entre los valores del GER medidos y estimados. La infraestimacion fue mayor en pacientes con insuficiencia pancreatica exocrina y en homocigoticos ΔF508.

OBJECTIVE Undernutrition, which implies an imbalance between energy intake and energy requirements, is common in patients with cystic fibrosis. The aim of this study was to compare resting energy expenditure determined by indirect calorimetry with that obtained with commonly used predictive equations in adults with cystic fibrosis and to assess the influence of clinical variables on the values obtained. PATIENTS AND METHODS We studied 21 patients with clinically stable cystic fibrosis, obtaining data on anthropometric variables, hand grip dynamometry, electrical bioimpedance, and resting energy expenditure by indirect calorimetry. We used the intraclass correlation coefficient (ICC) and the Bland–Altman method to assess agreement between the values obtained for resting energy expenditure measured by indirect calorimetry and those obtained with the World Health Organization (WHO) and Harris–Benedict prediction equations. RESULTS The prediction equations underestimated resting energy expenditure in more than 90% of cases. The agreement between the value obtained by indirect calorimetry and that calculated with the prediction equations was poor (ICC for comparisons with the WHO and Harris–Benedict equations, −; 0.47 and 0.41, respectively). Bland–Altman analysis revealed a variable bias between the results of indirect calorimetry and those obtained with prediction equations, irrespective of the resting energy expenditure. The difference between the values measured by indirect calorimetry and those obtained with the WHO equation was significantly larger in patients homozygous for the ΔF508 mutation and in those with exocrine pancreatic insufficiency. CONCLUSIONS The WHO and Harris–Benedict prediction equations underestimate resting energy expenditure in adults with cystic fibrosis. There is poor agreement between the values for resting energy expenditure determined by indirect calorimetry and those estimated with prediction equations. Underestimation was greater in patients with exocrine pancreatic insufficiency and patients who were homozygous for Δ F508.

Introduction: Chronic bronchial Pseudomonas (PA) infection is associated with increased exacerbations and hospitalizations in patients with non-CF bronchiectasis (NCFBC). Aims: To demonstrate that treatment with Colistin by I-neb® reduces exacerbations and hospitalizations, and that this is related with adherence to treatment and ability to use the device. Methods: Observational study comparing exacerbations and hospitalizations in the pre- and post-initiation of treatment with Colistin year, analyzing whether adherence and ability to use the I-neb® influence the results. Results: 19 patients (68.4% male) were collected with a median age of 70(21). 100% of patients had exacerbations in the previous year, median 5(6); compared with 73.7% in the following year, median 1(3), p Conclusions: Treatment with Colistin by I-neb® device reduces exacerbations and hospitalizations in patients with NCFBC and chronic PA infection. This effect on exacerbations is clearly related to adherence. The use of I-neb® is simple and helps proper drug administration in most patients.