Spanish Registry Seen to Capture Disease Scope, May Guide Research

Spanish Registry Seen to Capture Disease Scope, May Guide Research

The Spanish Bronchiectasis Registry (RIBRON) accurately reflects the characteristics of people with bronchiectasis in Spain, and may provide a solid basis for studies focused on multiple aspects of this disease, a study reported.

Registry analyses, for example, showed that around two-thirds of enrolled patients are women, and they tend to have milder disease forms compared with men. But both groups have similar infectious and therapeutic profiles, supporting future research into sex-specific differences that might better guide treatment, its researchers said.

Their study, “RIBRON: The Spanish online bronchiectasis registry. Characterization of the first 1912 patients,” published in the journal Archivos de Bronconeumología.

In recent decades, the Spanish Society of Pulmonology and Thoracic Surgery (SEPAR) has worked to expand overall scientific understanding of bronchiectasis and its treatment. SEPAR was also responsible for creating the country’s first nationwide bronchiectasis registry, which collected data from more than 2,000 patients being followed at 36 hospitals between 2002 and 2011.

In 2015, SEPAR established a new patient registry with the goal of creating a platform for follow-up data from each patient, which might give a more reliable readout of the natural evolution of bronchiectasis over time.

This initiative led to RIBRON, one of the largest bronchiectasis patient registries in the world. As of July 2019, RIBRON had gathered information on more than 2,300 patients being monitored at 43 hospitals across Spain.

“Given the wide participation by hospitals throughout the country, the analysis of these patient data is helping us paint an accurate picture of the general characteristics of bronchiectasis in Spain,” the researchers wrote.

Scientists at several of the country’s institutions reviewed and described the demographic and clinical features of patients included in RIBRON as of February 2019.

Of these 2,115 people, 203 were excluded from this analysis for lack of valid data, pending information, or for having been diagnosed with cystic fibrosis, another chronic lung disease. A total of 1,912 adults with bronchiectasis were included in the study.

Patients had a mean age of 67.6, with nearly half (43.8%) over 70 years of age. Most were women (63.9%), had never smoked (58.4%), and did not regularly consume alcohol (58%). Practically all (96.7%) were Caucasian.

Productive cough was the most common disease symptom (78.3%), and almost a third (29.2%) also had some degree of shortness of breath.

In most cases, bronchiectasis systems were first evident following an infection, and were considered to have a post-infectious origin (40.4%). In 18.5% of these patients, the disease was idiopathic, meaning it did not have a clear-cut cause.

After reviewing radiological data, the investigators found that bronchiectasis tended to affect mainly the lower lobes of the lungs, followed by the middle lobe.

Nearly half (44.8%) of the patients had chronic bronchial infections. Pseudomonas aeruginosa was the most common disease-causing microbe isolated from patients’ lungs (40.4%). In 25.6% of the cases, it was associated with chronic infections.

According to two measures of disease severity, more than half (60.4–69.2%) of the patients had mild bronchiectasis, and 10% or less had severe disease.

Most of these people (57.4%) had at least one bronchiectasis exacerbation each year, with 13.2% hospitalized for flares at least once per year.

Three-fourths (75%) of the patients were on chronic corticosteroid treatment (primarily inhaled forms), while 77% were being treated with bronchodilators — medications that help to open the lungs’ airways, making it easier to breathe. More than half (60.2%) were also following a respiratory physiotherapy program, and 19.2% were on chronic treatment with macrolide antibiotics.

A further analysis revealed that women tended to be younger, and to have fewer symptoms and co-existing conditions (comorbidities) than men. Female patients also tended to have less severe forms of bronchiectasis, with fewer exacerbations requiring hospitalization.

However, the investigators found no differences between sexes regarding the frequency of mild-to-moderate exacerbations, the use of antibiotics or anti-inflammatory medications to control infections, or the radiological distribution of the disease.

“This analysis of the Spanish Bronchiectasis Registry (RIBRON) that includes nearly 2,000 patients paints an accurate picture of the characteristics of bronchiectasis in our country, and may be the basis for the implementation of multiple studies on this disease,” the researchers wrote.

“Two-thirds of patients are women, and their characteristics, especially clinical features, differ widely from those of men, so sex-specific studies are important in determining whether women need more personalized treatment and monitoring,” they added.