September 10, 2012 (Vienna, Austria) — An easy-to-use 7-point scale to evaluate disease severity in patients with noncystic fibrosis bronchiectasis has been developed. In a validation study, the score was significantly associated with mortality in a large series of patients and showed good specificity.
Results were presented here at the European Respiratory Society 2012 Annual Congress.
"Bronchiectasis is the third most frequent chronic disease of airways and is an irreversible dilation of the airway due to chronic infection or inflammation," Miguel Ángel Martinez-Garcia, MD, from the pneumology unit at the General Hospital of Requena in Valencia, Spain, noted in an interview with Medscape Medical News.
He emphasized that bronchiectasis is a multifaceted disease and that disease severity and patient outcome are often difficult to assess. A subjective grading system could be useful in evaluating the extent of the disease and in determining which patients require closer monitoring.
Multifaceted Disease, Multifaceted Scoring System
"When a patient with an airway disease comes to our outpatient clinic, it is important to know the severity of the disease to plan treatment," Dr. Martinez-Garcia said. "Unfortunately, we have not one single variable to explain the whole impact of the disease, since each has a separate etiology and progression rate. For example, patients with a severe cough may have only [limited] bronchiectasis or have no exacerbation, and there are patients with [extensive] bronchiectasis without symptoms. [We] developed a grading system to better define the severity of these diseases. Our objective was to construct a scale using information from one of the largest databases of patients with bronchiectasis in Europe," he reported.
This multicenter study involved 819 patients diagnosed with noncystic fibrosis bronchiectasis using high-resolution computed tomography. Of these patients, 397 were randomly selected to serve as the construction cohort (used to configure the test variables) and the remaining 422 served as the validation cohort (used to determine the sensitivity and specificity of the test). The mean age of the patients was 58.7 years and the cohort was 56% female. Patient characteristics were well balanced between the 2 groups.
The researchers constructed a 7-point score for disease severity, dubbed the FACED score, using the relative weight of 5 variables associated with the 5-year all-cause mortality of bronchiectasis: lung function, age, radiologic extension, microbiologic data, and symptoms.
In assessing lung function, postbronchodilation forced expiration volume in 1 s (FEV1) below 50% is assigned 0 points and an FEV1 above 50% is assigned 2 points. FEV1 had an odds ratio [OR] of 5.2 (95% confidence interval [CI], 2.8 to 9.8).
In assessing age, patients 70 years and younger are assigned 0 points and those older than 70 years are assigned 2 points (OR, 4.9; 95% CI, 2.7 to 9.3).
In assessing radiologic extension (OR, 1.9; 95% CI, 1.1 to 3.5), the involvement of only 1 lobe is assigned 1 point and the involvement of 2 or more lobes is assigned 2 points.
In assessing microbiologic data, the absence of Pseudomonas aeruginosa colonization (OR, 2.4; 95% CI, 1.3 to 4.6) is assigned 0 points and the presence is assigned 1 point.
In assessing symptoms (OR, 2.8; 95% CI, 1.5 to 5.2), the absence of dyspnea is assigned 0 points and the presence is assigned 1 point.
"Patients with 0, 1, or 2 points have mild bronchiectasis because their probability of death in the next 5 years from the diagnosis of bronchiectasis is less than 5%," Dr. Martinez-Garcia explained. "Patients with 3, 4, or 5 points have moderate bronchiectasis and patients with 6 or 7 points have severe bronchiectasis because their probability of dying within 5 years is almost 70%," he said. He added that the scale has good specificity.
In an interview with Medscape Medical News, Conway Wong, CMDHB, from the Department of Respiratory Medicine at Middlemore Hospital in Auckland, New Zealand, said that "this study has determined which factors best predict mortality in bronchiectasis."
It might be useful to incorporate this approach in "future clinical trials to determine if an intervention is more effective in patients with severe disease. It may also guide clinicians in deciding whether patients require more intensive treatment or monitoring," Dr. Wong added.
"In the next months, we will contact some colleagues worldwide with large databases in bronchiectasis to perform an external validation of the FACED score. We are awaiting publication of this study [before we begin to] use the scale in patients entering our clinic," Dr. Martinez-Garcia said. "This is an easy-to-use grading system" in which the construction and validation cohorts compared well, as did the different centers involved in the study, he noted.
Regarding future plans, he reported that because "age is not a treatable variable, we are studying a similar scoring system that does not include age but includes severe exacerbations."
This study was funded by Praxis Pharmaceutical and a grant from the Societad de Valenciana Neumologia. Dr. Martinez-Garcia and Dr. Wong have disclosed no relevant financial relationships.
European Respiratory Society (ERS) 2012 Annual Congress: Abstract LBA 2811. Presented September 3, 2012.
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Cite this: Jenny Powers. Bronchiectasis Grading System Identifies At-Risk Patients - Medscape - Sep 10, 2012.