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The SEPAR Spanish Bronchiectasis Registry (RIBRON) began as a platform for the collection of longitudinal data on patients with this disease. The objective of this study is to describe its operation and to analyze the characteristics of bronchiectasis patients according to sex.

Both systemic inflammation and exacerbations have been associated with greater severity of bronchiectasis. Our objective was to analyze the prognostic value of the peripheral concentration of C-reactive protein (CRP) for the number and severity of exacerbations in patients with bronchiectasis.

BACKGROUND: Health-related quality of life (QoL) is one of the most important end points in bronchiectasis (BE). However, the majority of health-related QoL questionnaires are timeconsuming or not validated in BE. The COPD Assessment Test (CAT) is an easy-to-use questionnaire. The objective of this study was to perform a complete validation of the CAT in BE. METHODS: This was an observational, multicenter, prospective study in patients with BE. Psychometric properties of the CAT were measured: internal consistency (Cronbach alpha), repeatability (test-retest; intraclass correlation coefficient), discriminant validity (correlation with severity scores), convergent validity (correlation with some validated QoL questionnaire and other clinical variables of interest), longitudinal validity (measuring before and after each exacerbation during follow-up to determine the sensitivity to change and responsiveness), predictive validity to future exacerbations, and finally minimum clinically important difference. RESULTS: Ninety-six patients were included and followed up for 1 year. Their mean age was 62.2 (15.6) years (79.2% women). The CAT showed excellent internal consistency (alpha, 0.95) and repeatability (intraclass correlation coefficient, 0.95). The validity of the CAT was excellent in all the measures (almost all with a Pearson coefficient > 0.40) except for the correlations with severity scores (Pearson coefficient between 0.22 and 0.26). Sensitivity to change before and after exacerbations was set at between 5.4 and 5.8 points. A CAT value >= 10 points showed prognostic value for patients with more than one exacerbation, and finally the minimum clinically important difference was set at 3 points. CONCLUSIONS: The CAT presented excellent psychometric properties and is a questionnaire that is easy to use and interpret in patients with BE.

Pseudomonas aeruginosa ( P. aeruginosa ) is a ubiquitous and opportunistic microorganism and is considered one of the most significant pathogens that produce chronic colonization and infection of the lower respiratory tract, especially in people with chronic inflammatory airway diseases such as asthma, chronic obstructive pulmonary disease (COPD), cystic fibrosis (CF), and bronchiectasis. From a microbiological viewpoint, the presence and persistence of P. aeruginosa over time are characterized by adaptation within the host that precludes any rapid, devastating injury to the host. Moreover, this microorganism usually develops antibiotic resistance, which is accelerated in chronic infections especially in those situations where the frequent use of antimicrobials facilitates the selection of "hypermutator P. aeruginosa strain". This phenomenon has been observed in people with bronchiectasis, CF, and the "exacerbator" COPD phenotype. From a clinical point of view, a chronic bronchial infection of P. aeruginosa has been related to more severity and poor prognosis in people with CF, bronchiectasis, and probably in COPD, but little is known on the effect of this microorganism infection in people with asthma. The relationship between the impact and treatment of P. aeruginosa infection in people with airway diseases emerges as an important future challenge and it is the most important objective of this review.

Background: The Cystic Fibrosis Questionnaire-Revised (CFQ-R+ 14) is a disease-specific, health-related quality of life instrument for cystic fibrosis (CF) patients >= 14 years. We have developed a Spanish electronic version of the CFQ-R (e-CFQ-R+ 14 Spain). Our aim was to compare the paper and electronic versions and to validate the electronic version. Methods: Fifty CF patients completed the study. All answered the paper and electronic versions on day 1 and repeated the e-CFQR version 15 days later. Results: Concordance between the electronic and paper copy versions was high, with correlations above 0.9 in all domains. Test-retest reliability of the e-CFQ-R results was strong, with coefficients ranging from 0.8 to 0.9. Conclusions: The e-CFQ-R version is reliable and valid and can replace the paper copy, thus simplifying the assessment of quality of life. It also provides immediate results with no errors in scoring. It is a useful new tool in CF care. (C) 2017 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

The first Spanish multi-centre study on the microbiology of cystic fibrosis (CF) was conducted from 2013 to 2014. The study involved 24 CF units from 17 hospitals, and recruited 341 patients. The aim of this study was to characterise Pseudomonas aeruginosa isolates, 79 of which were recovered from 75 (22%) patients. The study determined the population structure, antibiotic susceptibility profile and genetic background of the strains. Fifty-five percent of the isolates were multi-drug-resistant, and 16% were extensively-drug-resistant. Defective mutS and mutL genes were observed in mutator isolates (15.2%). Considerable genetic diversity was observed by pulsed-field gel electrophoresis (70 patterns) and multi-locus sequence typing (72 sequence types). International epidemic clones were not detected. Fifty-one new and 14 previously described array tube (AT) genotypes were detected by AT technology. This study found a genetically unrelated and highly diverse CF P. aeruginosa population in Spain, not represented by the epidemic clones widely distributed across Europe, with multiple combinations of virulence factors and high antimicrobial resistance rates (except for colistin).

Background: Clinical and demographical knowledge on Spanish cystic fibrosis (CF) patients is incomplete as no national registry exists. CF-microbiology has not been studied at national level. The results of the first Spanish multicenter study on CF microbiology are presented. Methods: 24 CF-Units for adult (n = 12) and pediatric (n = 12) patients from 17 hospitals provided sputa and clinical data from 15 consecutive patients. Cultures and susceptibility testing were performed. Colonization impact on pulmonary function was assessed. Results: 341 patients [mean (SD) age 21 (11) years, 180 >= 18 years, mean (SD) FEV1 = 68 (25)%] were included. Pseudomonas aeruginosa was reported as chronic, intermittent or absent in 46%, 22% and 32% of patients, respectively. The annual prevalence was 62%. Positive P. aeruginosa and methicillin-resistant Staphylococcus aureus cultures were significantly associated with lower FEV1 (p < 0.001 and p = 0.003, respectively). Conclusions: The representative subset of the Spanish CF-population which has been clinically, demographically and microbiologically. characterized will serve as a reference for future CF studies in Spain. (C) 2015 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

RATIONALE: The clinical presentation and prognosis of non-cystic fibrosis bronchiectasis are both very heterogeneous. OBJECTIVES: To identify different clinical phenotypes for non-cystic fibrosis bronchiectasis and their impact on prognosis. METHODS: Using a standardized protocol, we conducted a multicenter observational cohort study at six Spanish centers with patients diagnosed with non-cystic fibrosis bronchiectasis before December 31, 2005, with a 5-year follow-up from the bronchiectasis diagnosis. A cluster analysis was used to classify the patients into homogeneous groups by means of significant variables corresponding to different aspects of bronchiectasis (clinical phenotypes): age, sex, body mass index, smoking habit, dyspnea, macroscopic appearance of sputum, number of exacerbations, chronic colonization with Pseudomonas aeruginosa, FEV1, number of pulmonary lobes affected, idiopathic bronchiectasis, and associated chronic obstructive pulmonary disease. Survival analysis (Kaplan-Meier method and log-rank test) was used to evaluate the comparative survival of the different subgroups. MEASUREMENTS AND MAIN RESULTS: A total of 468 patients with a mean age of 63 (15.9) years were analyzed. Of these, 58% were females, 39.7% had idiopathic bronchiectasis, and 29.3% presented with chronic Pseudomonas aeruginosa colonization. Cluster analysis showed four clinical phenotypes: (1) younger women with mild disease, (2) older women with mild disease, (3) older patients with severe disease who had frequent exacerbations, and (4) older patients with severe disease who did not have frequent exacerbations. The follow-up period was 54 months, during which there were 95 deaths. Mortality was low in the first and second groups (3.9% and 7.6%, respectively) and high for the third (37%) and fourth (40.8%) groups. The third cluster had a higher proportion of respiratory deaths than the fourth (77.8% vs. 34.4%; P < 0.001). CONCLUSIONS: Using cluster analysis, it is possible to separate patients with bronchiectasis into distinct clinical phenotypes with different prognoses.

Pseudomonas aeruginosa is the main pathogen in bronchopulmonary infections in cystic fibrosis (CF) patients. It can only be eradicated at early infection stages while reduction of its bacterial load is the therapeutic goal during chronic infection or exacerbations. Neonatal screening and pharmacokinetic/pharmacodynamic knowledge has modified the management of CF-patients. A culture based microbiological follow-up should be performed in patients with no infection with P. aeruginosa. At initial infection, inhaled colistin (0,5-2 MU/tid), tobramycin (300 mg/bid) or aztreonam (75 mg/tid) with or without oral ciprofloxacin (15-20 mg/kg/bid, 2-3 weeks) are recommended. In chronic infections, treatment is based on continuous administration of colistin or with a 28-day on-off regimen with tobramycin or aztreonam. During mild-moderate exacerbations oral ciprofloxacin (2-3 weeks) can be administered while serious exacerbations must be treated with intravenous combination therapy (beta-lactam with an aminoglycoside or a fluoroquinolone). Future studies will support antibiotic rotation and/or new combination therapies. Epidemiological measures are also recommended to avoid new P. aeruginosa infections and "patient-to-patient transmission" of this pathogen. (C) 2014 SEPAR. Published by Elsevier Espana, S.L.U. All rights reserved.

Background: Information on the role of fungi in non-cystic fibrosis (CF) bronchiectasis is lacking. Objectives: Our aim was to determine the prevalence of and factors associated with the isolation and persistence of fungi from sputum in these patients. Methods: We performed a multicenter observational study comprising adult patients with non-CF bronchiectasis. Persistence of Aspergillus spp. and Candida albicans was defined as the presence of >= 2 positive sputum cultures taken at least 6 months apart within a period of 5 years. Results: A total of 252 patients (62.7% women with a mean +/- SD age of 55.3 +/- 16.7 years) were included in the study. All patients had at least 1 sputum sample cultured for fungi, with a mean +/- SD of 7 +/- 6 cultures per patient. Eighteen (8.7%) and 71 (34.5%) patients had persistent positive cultures for Aspergillus spp. and C. albicans, respectively. Patients with persistence of Aspergillus spp. and C. albicans were older and had more daily purulent sputum. In addition, patients with persistent C. albicans had worse postbronchodilator forced expiratory volume in the first second (FEV1), more frequent cystic bronchiectasis, and more hospital-treated exacerbations. They were also more frequently treated with long-term antibiotics. Multivariate analysis showed that daily purulent sputum (OR = 3.75, p = 0.045) and long-term antibiotics (OR = 2.37, p = 0.005) were independently associated with persistence of Aspergillus spp. and C. albicans, respectively. Conclusions: Isolation and persistence of Aspergillus spp. and C. albicans are frequent in patients with non-CF bronchiectasis. Daily purulent sputum and chronic antibiotic treatment were associated with persistence of Aspergillus spp. and C. albicans, respectively. (C) 2015 S. Karger AG, Basel

Background: The aim was to measure symptoms of depression and anxiety in patients with bronchiectasis and evaluate their relationship with a Mediterranean diet. Methods: This cross-sectional study recruited patients with bronchiectasis at four Spanish centers. Patients completed the hospital anxiety and depression scale (HADS) and the Mediterranean diet questionnaire (PREDIMED). Demographic, health and outcome data were recorded from medical charts. Logistic regression was used to determine the predictors of elevated symptoms of depression and anxiety (HADS >= 11). Results: Of the 205 participants recruited, 37 (18.0%) had elevated anxiety-related scores and 26 (12.7%) had elevated depression-related scores (HADS >= 11). Increased symptoms of depression were significantly associated with being unemployed, a lower education, older age, comorbidity, major dyspnea, worse quality of life (QOL) and a lower PREDIMED score. Increased symptoms of anxiety were significantly associated with more exacerbations, worse QOL and a lower PREDIMED score. Regression analyses indicated that, after adjustment, QOL and the PREDIMED score predicted elevated symptoms of depression and QOL predicted elevated symptoms of anxiety. Conclusions: The prevalence of elevated symptoms of depression and anxiety is high in patients with bronchiectasis and greater adherence to a Mediterranean diet is associated with a lower likelihood of having these symptoms, particularly for depression. (C) 2014 Elsevier Inc. All rights reserved.