September 10, 2012 (Vienna, Austria) DD researchers have developed an easy-to-use 7-point scale for evaluating disease severity in patients with non-cystic fibrosis bronchiectasis. Validation studies have shown that the score correlates significantly with mortality in large series of patients with good specificity. The results of the study were presented right at the 2012 European Respiratory Society Annual Meeting. ”Bronchiectasis is the third most common chronic disease of the respiratory tract and is an irreversible dilatation of the airways caused by chronic infection or inflammation.” Dr. Miguel ángel Martinez-Garcia, of the Pulmonology Department at Requena General Hospital in Valencia, Spain, noted in an interview with Medscape Medical News. He emphasized that bronchiectasis is a multifaceted disease whose severity and patient outcomes are often difficult to assess. A subjective grading system can be useful in evaluating the extent of the disease and determining which patients need to be monitored more closely. Multifaceted disease, multifaceted grading system “When a patient with respiratory disease comes to our clinic, it is important to know the severity of the disease so that a treatment plan can be developed,” says Dr. Martinez-Garcia. “Unfortunately, because each variable has its own etiology and progression rate, we don’t yet have a univariate system that accounts for the overall impact of the disease. For example, patients with severe cough may have only (limited) bronchiectasis, or no exacerbations; some patients have (extensive) bronchiectasis but no symptoms. (We) developed a grading system to better define the severity of these diseases. Our aim was to set up a scale using information from one of the largest databases of patients with bronchiectasis in Europe,” he reported. The multicenter study included 819 patients with non-cystic fibrosis bronchiectasis diagnosed by high-resolution CT, of whom 397 were randomly selected as the setting cohort (for calculating test variables) and the remaining 422 were part of the validation cohort (for judging the sensitivity and specificity of the test). The mean age of the patients was 58.7 years, and women comprised 56% of the cohort. Patient characteristics were adequately balanced between the two groups. The investigators set a 7-point scale of disease severity, called the FACED scale, using relative weights of one variable associated with 5-year all-cause mortality in bronchiectasis: lung function, age, radiographic range, microbiological profile, and symptoms. Pulmonary function was assessed: a score of 0 for forceful expiratory volume in 1 second after bronchodilation (FEV1) <50%, a score of 1 for FEV1 >50%, and an FEV1 ratio (OR) of 5.2 (95% confidence interval [CI], 2.8 – 9.8). Age assessment: 0 for patients ≤70 years and 2 for patients >70 years (OR, 4.9; 95% CI, 2.7-9.3). Radiographic range assessment (OR, 1.9; 95% CI, 1.1 – 3.5): 1 point for 1 page of irradiation and 2 points for ≥2 pages of irradiation. Microbiological profile assessment: 0 points for the absence of P. aeruginosa (OR, 2.4; 95% CI, 1.3 – 4.6) and 1 point for the presence. Symptom assessment (OR, 2.8; 95% CI, 1.5 – 5.2): 0 for no dyspnea and 1 for yes. ”Patients with a score of 0, 1 or 2 had mild bronchiectasis because the probability of death in the next 5 years from the diagnosis of bronchiectasis was less than 5%,” Dr. Martinez-Garcia explained, “and patients with a score of 3, 4 or 5 had moderate bronchiectasis. Patients with a score of 6 or 7 have severe bronchiectasis, because the probability of death in 5 years in these patients is almost 70 percent,” he said. He then added that the scale has good specificity. In an interview with Medscape Medical News, Dr. Conway Wong of the Department of Respiratory Medicine at Middlemore Hospital in Auckland, New Zealand, said, “The current study determines which factors have the best prediction of bronchiectasis mortality.” It may be useful for incorporating this approach into “future clinical trials to determine whether interventions are effective in patients with severe disease. It may also serve as a guide for clinicians when deciding whether patients need more extensive treatment or monitoring,” Dr. Wong said. ”In the coming months, we will be contacting colleagues around the world with large bronchiectasis databases to conduct external validation of the FACED scale. We will then (begin) using this scale on outpatients after the results of this study are published,” Dr. Martinez-Garcia said. “It’s an easy-to-use grading system,” with a good setup and validation cohort match, and a good fit between the different research centers involved in the study, he emphasized. As for future plans, he reported that because “age is an untreatable variable, we are working on a similar scoring system that will not include age, but will include the number of severe exacerbations.”