RIBRON is a centralized database of bronchiectasis patients from 43 hospitals located throughout Spain. The objectives of this record are:

• 1

  To obtain information from bronchiectasis patients to improve the knowledge of the disease in our country.

• 2

  To facilitate and promote multicenter and multidisciplinary research in bronchiectasis.

• 3

  To identify groups of patients who may be candidates for future clinical trials.

• 4

  To assess the follow-up of Spanish bronchiectasis clinical guidelines and recommendations in the attempt to standardize and improve patient management.

The inclusion criteria are as follows: patients over 18 years of age with bronchiectasis of any etiology (including cystic fibrosis [CF], although only non-CF bronchiectasis will be considered for this analysis). All patients should be diagnosed with chest high-resolution computed tomography (HRCT) and have a clinical picture consistent with the disease. Traction bronchiectasis was excluded. All ethics committees in the participating centers approved the registry, and all patients signed an informed consent to include their data in the registry. The recommendations of the Helsinki Declaration, good clinical practice guidelines, and currently applicable data protection laws were followed at all times. The registry is sponsored by SEPAR.

Data entry for each patient consists of a broad set of variables composed of 637 baseline and annual follow-up items, of which 79 were specified as basic or critical. These were defined as the minimum variables that, given their relevance, had to be entered for patients to be considered valid. These variables comprise general and anthropometric data, diagnostic variables, etiology and complementary etiological tests, disease history and comorbidities, clinical picture and physical examination, lung function, basic laboratory tests (in addition to those specific for etiological diagnosis), radiological diagnosis, microbiological data, exacerbations, complications, and treatments (pharmacological and non-pharmacological) both in stable phase and during exacerbations. In addition to these variables, an annual follow-up section is completed which lists any new circumstances that have emerged since the last visit, encompassing all the aspects mentioned above.

A company not affiliated with RIBRON and independent from researchers and SEPAR is responsible for data quality control. To this end, various measures have been established:

• 1

  In all variables, values entered outside the reasonable preset ranges trigger an alert. In this case, the the investigator is notified that the datum cannot be entered.

• 2

  If a confusing or conflicting data point is detected, the company contacts the investigator to certify the authenticity of the entry.

• 3

  An annual database sweep is performed for abnormal data or missing data. The corresponding researchers are informed of the findings in an external audit that is recorded, and a report is generated and forwarded to the registry scientific committee

• 4

  Validation is performed individually for each patient.

• 5

  A data dump is performed annually in SPSS® or Excel® format and the content is purged by a professional statistician.

• 6

  The company ensures the confidentiality of the data according to applicable legislation.

This study analyzed the characteristics of 1,912 patients from 43 hospitals throughout Spain in terms of general and anthropometric data, diagnostic variables, etiology and complementary etiological tests, disease history and comorbidities, clinical picture and physical examination, lung function, radiological diagnosis, microbiological data, exacerbations, and treatments (pharmacological and non-pharmacological). Variables are tabulated by mean (standard deviation) for variables with normal distribution, median (interquartile range) for non-normal distribution, and by percentage of total for qualitative or dichotomous variables. A Mann-Whitney U/Student’s t test or Chi-squared test (depending on the type and distribution of variables) was used to analyze the differential characteristics between groups according to sex. A p-value of <0.05 was considered statistically significant. The data were analyzed using the SPSS® statistical package version 21.0.

Mean age was 67.6 years (15.2), 43.8% were over 70 years of age, and there was a predominance of women (63.9%). In total, 96.7% were Caucasian. The diagnostic delay was 6.2 years (12.1). Body mass index (BMI) was 25.6 kg/m2 (4.8) (Table 1).

Most patients had never smoked (58.4%), and did not consume alcohol (58%). Among smokers and former smokers, the number of pack-years was 31.4 (27.6); 8.3% were active smokers and 33.3% were former smokers. The mean Charlson index was 1.83 (1.46).

The most commonly reported disease history was nasal rhinosinusitis/polyposis, which was present in 27.7%, followed by cardiovascular disease (17.4% of patients had at least one previous cardiovascular event), and tumor disease (7.2%). Between 7.4% and 8.4% of patients were diagnosed with anxiety or depression, but more than a third of the overall group of study patients (34.9%) were receiving at least one psychoactive drug (Table 1).

The most frequent symptom was cough (78.3%) with expectoration (69.6%). Overall, 29.2% of patients had at least grade II/IV dyspnea (mMRC) (Table 1).

Fig. 2 shows the distribution of patients according to the number and severity of exacerbations during the year before and the year after inclusion in the registry. A mean of 1.62 (1.9) mild-moderate exacerbations and 0.59 severe exacerbations (need for hospitalization or intravenous antibiotic therapy in hospital or at home due to exacerbation) occurred in the year before inclusion,1,3 and 1.551,7 and 0.571,2 in the year following inclusion, respectively. No significant differences were found in the number or severity of exacerbations between those occurring in the year before and the year after inclusion in the registry. Overall, 57.4%, 19.5%, and 10.9% of patients had at least 1, 2, or 3 exacerbations per year, while 13.2% had 1 or more hospital stays per year. Using a definition of an exacerbator as a patient with at least 2 exacerbations per year or 1 hospitalization per year 16 22.4% of the series could be considered as exacerbators.

Fig. 2.

Distribution of the number of mild-moderate (A) and severe (B) bronchiectasis exacerbations in the year preceding and following inclusion in the registry.

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Valid etiological data were available from 99.4% of patients. Table 2 lists the most common etiologies by groups. The most frequent etiology was post-infectious disease (40.4%), while 18.5% of patients presented bronchiectasis defined as idiopathic.

Mean baseline FEV1 was 1.86 l (0.8) (73.9% predicted), while mean forced vital capacity (FVC) was 2.66 (0.9) (83.8% predicted). In total, 53.1% had airflow obstruction defined as an FEV1/FVC ratio < 0.7, while 17% had severe airflow obstruction (FEV1 < 50%), and 19.8% of patients had a positive bronchodilator test. Six-minute walk test data were available from 131 patients who walked an average of 425.3 m (114). On average, alveolar volume-corrected carbon monoxide diffusion values (DLCO/VA) were found to be within normal parameters: 81%. Mean arterial blood gases showed moderate hypoxemia in blood gases (pO2: 66 mmHg [17.5] and pCO2: 39.7 mmHg [6.7]). Data were available from 355 patients with lung volumes showing a mean total lung capacity (TLC) of 6.43 l (3.1) (102% predicted) and a residual volume (RV) of 3.78 l (11.5) (138% predicted).

Fig. 3 shows the distribution of bronchiectasis by pulmonary lobes. The most frequent locations were the lower lobes (64.7–56.9%) and the middle lobe (56.9%). Bronchiectasis in more than 2 pulmonary lobes was observed in 48.2%, 90.9% of which were cylindrical. Varicose bronchiectasis was detected in 26.4% of the patients and cystic bronchiectasis in 21%. In addition, 19.4% presented radiological images consistent with bronchiolitis (“tree-in-bud” image).

Fig. 3.

Distribution of bronchiectasis by lobes.

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Mean FACED (17) and E-FACED (18) scores were 2.06 (1.69) and 2.67 (2.17), respectively, while the Bronchiectasis Severity Index (BSI) (19) was 7.8 (4.5). Fig. 4 shows the percentage by severity (mild, moderate, or severe) of the 3 multidimensional scores, and the graphical distribution of FACED and E-FED scores.

Fig. 4.

Distribution of severity based on FACED, E-FACED, and BSI scores.

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Table 3 summarizes the main microbiological characteristics of bronchiectasis patients. Overall, 38.5% had initial colonization, 16.7% intermittent, and 44.8% chronic bronchial infection. The most common potentially pathogenic microorganism (PPM) involved (both in single isolations and in the form of chronic bronchial infection [CBI]) was Pseudomonas aeruginosa (P. aeruginosa) with 40.4% and 25.6% of patients, respectively. Only atypical mycobacteria were isolated in 26 patients, the most frequent being Mycobacterium avium complex.

A total of 75% of patients were receiving chronic corticosteroids, of which 87% were inhaled (66.7% of all patients); 77% of patients were taking some type of bronchodilator treatment (67% short-acting beta 2; 68% long-acting beta 2; and 44% anticholinergics); 60.2% of patients followed a respiratory physiotherapy program, with manual secretion drainage techniques in 69% of cases; 8.2% used mucolytics and 2.5% hypertonic serum, mainly 7%; 7.4% of patients received home oxygen therapy, while 0.9% required non-invasive mechanical ventilation. Finally, 19.2% received chronic macrolides (93% azithromycin) and 19.5% inhaled antibiotics (78% of which were nebulized sodium colistimethate).

Table 4 summarizes the general characteristics of the patients by sex. Women had a very different disease profile from men, with a lower age, lower BMI, fewer symptoms, fewer comorbidities, and more mental disorders (anxiety/depression and taking psychoactive drugs). Bronchiectasis among women was due more to idiopathic forms, post-infectious disease, and asthma, and less to COPD. Disease severity was lower, they had fewer admissions for exacerbations, and their lung function was better. However, there were no differences in the number of mild-moderate exacerbations, the presence of CBI caused by P. aeruginosa, antibiotic or anti-inflammatory treatment of the infection, or radiological extension of the bronchiectasis.