Focus on Bronchiectasis/COPD Overlap: An Under-Recognized But Critically Important Condition Gary Hansen, PhD Former “Orphan Disease” Grows in Recognition Recognizing • Chronic productive cough Non-cystic fibrosis bronchiectasis (NCFB)1 is an important Bronchiectasis2 disease that remains largely unknown. Once thought to be a rare • Chronic mucus hypersecretion “orphan” condition—merely the aftermath of infectious diseases • Chronic antibiotic use that are now readily treated—emerging evidence now shows that NCFB is far more common than originally believed and is closely • Frequent hospitalizations associated with another better known major respiratory health • Reduced quality of life threat—COPD.3 Defined as the thickening and enlargement of the airways resulting from chronic inflammation, bronchiectasis damages normal airway clearance mechanisms and results in the The process is irreversible and there is no known cure— accumulation of excess secretions. As a result, patients face fortunately, appropriate care can substantially improve the chronic cough, excess sputum production, and a recurring cycle lives of affected patients. of hospitalizations.2 Appropriate diagnosis and treatment are key to improving quality of life and reducing the need for expensive While bronchiectasis may be found at any age, it is most often medical care. Specifically, airway clearance therapy directly a disease of middle-age and older. The profile of a patient with addresses the problem of purulent sputum retained in the bronchiectasis is in many ways similar to that of chronic airways and by “emptying the Petri dish” can help to reduce the bronchitis.8 Affected patients often have a chronic productive risk of future exacerbations without risking exposure to the cough for more than three months of the year along with negative effects of long-term orWhile rotating bronchiectasis antibiotics. may4 be found at any age,copious it is most sputum often production a disease of that middle is difficult-age and toolder fully. The clear. They 8 profile of a patient with bronchiectasis is inmay many have ways multiple similar to unsuccessful that of chronic attempts bronchitis. with Affected airway Bronchiectasis has rightly beenpatients called oftenan “orphan have a chronicwith many productive coughclearance for more techniques. than three As months a result, of the their year quality along withof life is often parents”5 because it is the late copiousstage of sputum a number production of pulmonary that is difficult poor to fully due clear. to cough, They may shortness have multiple of breath, unsuccessful and fatigue. attempts9 Typically, diseases and a disease with a pulmonarywith airway component.clearance techniques. Among As a result,by the their time quality they of are life diagnosed,is often poor patients due to cough, will have shortness received these are infectious diseases suchof breath, as tuberculosis and fatigue. and9 Typically, by the timemultiple they are courses diagnosed, of antibiotics patients will punctuated have received with multiple frequent nontuberculous mycobacteria courses(NTM), of severe antibiotics lower punctuated respiratory with frequentexacerbations, exacerbations, often often requiring requiring hospitalizations. hospitalizations. The The gold tract infections, chronic aspirationgold standard of gastric for contents, bronchiectasis inhaled diagnosis standardis the high -forresolution bronchiectasis CT scan, which diagnosis can show is the varying high-resolution degree CT foreign objects, and genetic conditionsof bronchial such wall as thickening cystic fibrosis, and dilation ofscan, the airways. which2 can It is showimportant varying to note degrees that patients of bronchial may have wall alpha-1 antitrypsin deficiency, radiographicand primary evidence ciliary dyskinesia.of bronchiectasis butthickening show no symptoms; and dilation indeed, of the they airways. may be2 stable It is important in the to note Autoimmune disorders, such ascondition rheumatoid for years. arthritis, However, the airway damagethat patients may develop may have into radiographicsignificant symptoms evidence at any of time sarcoidosis, and granulomatosisgiven with the polyangiitis appropriate havetrigger. also bronchiectasis but show no symptoms; indeed, they may be been linked to NCFB. Additionally, diseases that affect the stable in the condition for years. However, the airway damage immune system, for instance, HIVThe andCOPD common Connection variable may develop into significant symptoms at any time given the immunodeficiency, may lead to chronic or recurrent infections appropriate trigger. resulting in bronchiectasis in theToday susceptible a large and host. growing Despite number of patients with COPD also have been diagnosed with bronchiectasis. considerable effort, no identifiableMore causethan 20 is million found peoplein 30-50 in the U.S. 10 percent of cases.6 Once the airwayslive with are COPD distended; of these and more ciliary than 7.7 Million four million patients may be affected by 20 Million Diagnosable COPD Moderate-Very transport damaged, a “vicious cycle” will often begin: mucus is 10 Diagnosable COPD Severe10 retained in the airways becomingbronchiectasis, a site for bacterial yet only about 500,000 colonization; this in turn provokeshave anbeen inflammatory diagnosed with response the condition , a number rising at an annual7 rate of 8.7 4.2 Million that, if it becomes chronic, causes additional damage. The 12 percent, most likely due to increased Diagnosable Bronchiectasis progression may be slow or swift depending on a number of awareness and surveillance of the factors, but once begun, the patient faces an ongoing cycle of disease.11 A recent meta-analysis of 500 Thousand+ recurring infections, resulting in reduction in lung function Diagnosed Bronchiectasis19 available data found that over 50 and quality of life, and the likelihood of ongoing medical care. percent of patients with moderate-to- severe COPD also have evidence of Gary Hansen is the Senior Managerbronchiectasis. of Scientific Affairs,12 The RespirTech, causes, a FigureFigure 1. 1 EstimateEstimatess of of the the prevalence prevalence of of diagnosable diagnosable COPD, COPD, diagnosable Philips Company. treatments, and relationships diagnosablebronchiectasis, bronchiectasi and diagnoseds, and diagnosedbronchiectasis. bronchiectasis. between comorbid conditions within COPD are controversial and a subject of intensive research. The evidence has led some researchers to Respiratory Therapy Vol. 13 No. 4 n Fall 2018 55 propose a “COPD-bronchiectasis overlap syndrome,”13 while this concept is still debatable.14 Nonetheless, COPD may be considered a possible cause of bronchiectasis15, and bronchiectasis is certainly an exacerbating factor in COPD.16 It has long been known that COPD patients often experience a cycle of exacerbation followed by temporary recovery. More recently, a study by Suissa et al. has shown that exacerbations follow a distinct pattern: after the first event, each subsequent exacerbation follows within a shorter time and tends to be more severe than the last.17 In this study, risk of a severe exacerbation increased three times after the first exacerbation, and 24 times after the tenth. This finding emphasizes the importance of early intervention in recognizing and treating the disease, particularly when combined with bronchiectasis. A recent meta-analysis by Du et al., of 5,329 COPD patients found a greatly increased exacerbation risk due to comorbid COPD with bronchiectasis compared to COPD alone.18 The risk of exacerbations rose almost two times higher, colonization of the lungs four times higher, severe airway obstruction 30 percent higher, and mortality two times higher. It is not surprising that these elevated risks are also associated with higher healthcare costs. A recent study found that compared to COPD alone, COPD + bronchiectasis resulted in 32 percent more hospitalizations and 27 percent higher hospitalization costs.19 With these issues at stake, there is a clear need to focus on these at-risk The COPD Connectionpatients. New treatment guidelines for bronchiectasis have recently been Today a large and growing number of patients with COPD also released by the European Respiratory Society (ERS).4 This have been diagnosedNew Guidelines with bronchiectasis. for Treatment More than 20 million important report contains a broad survey of available evidence, people in the US live with COPD10; of these more than four including drug treatments and a discussion of airway clearance. million patients may be affected by bronchiectasis, yet only The ERS guidelines state that “before considering the about 500,000Given have been that diagnoseda sizable withfraction the condition, of COPD apatients whoprescription are troubled of long-term with excess antibiotics, sputum general production aspects ofmay number risingharbor at an annual bronchiectasis, rate of 8.7 percent, it seem mosts prudent likely due to evaluatebronchiectasis for the condition management using need a high to be-resolution optimized suchCT scan as airway. to increased awarenessAlong with and bronchodilators surveillance of the and disease. mucolytics,11 A bronchiectasisclearance.” Thepatients list of are airway sometimes clearance treated methods with includes long - recent meta-analysisterm antibiotics of available, such data foundas macrolides that over .50 While usefulpulmonary in addressing physiotherapy, severe exacerbations, oscillating positive long expiratory-term