Bronchiectasis in adult patients is frequently misdiagnosed, yet is associated with increased morbidity, and increased utilization of medical care. Patients typically present with a chronic cough, usually productive with thick sputum on most days. Frequently they are misdiagnosed with recurrent bronchitis, COPD, and asthma. The prevalence of bronchiectasis increases with age, and is more common in women. In previous studies, it was estimated that there are about 110,000 individuals with bronchiectasis in the United States alone.1
The development of bronchiectasis occurs typically after infection, followed by an abnormal immune system response or impaired bronchial drainage. Impaired immune response and healing pathways typically lead to progressive bronchial inflammation and ultimately permanent destruction of the normal bronchial walls with subsequent dilation of the airways. The dilation of the airway leads to further impairment of normal bronchial drainage, and allows the patient to develop recurrent infections. There is a significant increase in neutrophilic inflammation found in the airway.2 It is not known whether decreased phagocytosis is partly responsible for the impaired immune response, or if higher levels of elastase released by neutrophils precipitates the damage3. Both of these pathways, however, are affected by the presence of alpha-1 antitrypsin, which may help explain why patients who are deficient have a higher incidence of bronchiectasis.
In North America, the most common etiologies for bronchiectasis are: connective tissue disease, allergic bronchopulmonary aspergillosis, immunodeficiency states, aspiration, non-tuberculous bacterial infection, and hematologic malignancy.4 Any obstruction of the airway may lead to bronchiectasis as well. This may occur from an aspirated foreign body, external compression of a bronchus, or an intraluminal lesion. Patients with hypogamaglobulinema or IgG subclass deficiencies may also develop bronchiectasis. Typically they have a history of recurrent sinopulmonary infections, often since childhood. Adults found to have IgG subclass deficiencies typically require antigen challenges and monitoring of antibody titers to confirm a true deficiency. Prolonged immunosuprresion also predisposes patients to bronchiectasis. The connective tissue diseases most likely to be associated with bronchiectasis are rheumatoid arthritis, and Sjögren syndrome, but is occasionally seen in other related diseases such as inflammatory bowel disease.
Certain infections are associated with the development of bronchiectasis. Non-tuberculous mycobacterial infections commonly are found as secondary infections in patients with bronchiectasis. However, it is found in otherwise healthy individuals, and may be a direct cause of bronchiectasis. It does have a predilection for women over 50 years of age, typically non-smokers. Mycobacterium avium complex (MAC) is the most common of these infections. Allergic bronchopulmonary aspergillosis is also associated with bronchiectasis. Typically these patients are treated for refractory asthma for many years and the sputum is typically thick and often forms plugs. These patient often have eosinophilia and very high serum IgE levels. Pertussis (Whooping cough) in children, historically, was a significant risk factor, however with vaccination this has been significantly reduced.
Genetic related diseases are also associated with the development of bronchiectasis. Examples include: primary ciliary dyskinesia, Kartagener’s syndrome (bronchiectasis, sinusitis, and dextrocardia), alpha-1 antitrypsin deficiency, cystic fibrosis, Young’s syndrome (bronchiectasis, sinusitis, and obstructive azoospermia), and autosomal dominant polycystic kidney disease. Although some diseases are diagnosed in infancy or childhood, some may not present until later in life. It is estimated that 7% of cystic fibrosis patients are diagnosed after 18 years of age.5
The most common symptom is a chronic cough, followed by daily sputum production. Dyspnea, rhino-sinusitis (frequently with loss of smell), and hemoptysis are common as well. The patient will typically have crackles in the affected region, and may have wheezing.
A chest radiograph may confirm the presence of bronchiectasis. Patients with abnormal but non-diagnostic chest radiographs, should undergo computed tomography. High resolution computed tomography (HRCT) is the best imaging modality available. It will confirm the presence of bronchiectasis, help in evaluating the extent of the disease, and will reveal other confounding diseases (ie. emphysema, interstitial lung disease, malignancy, or airway obstruction).
Further evaluation of bronchiectasis should include a complete blood count and differential, immunoglobulin quantitation and subclass analysis, and sputum cultures for bacteria, fungus, and mycobacterium. Pulmonary function testing and six minute walk testing help assess the functional status of the patient. Obstructive findings are the most frequent finding on pulmonary function testing. Other testing is performed based on the suspected underlying disease, such as sweat chloride testing, bronchoscopy, and nasal mucosal biopsy.
Once bronchiectasis has been established, treatment is directed to minimize the risk of future infections and improve quality of life. Sputum clearance measures and reduction of bacterial burden are the mainstay of therapy. Mucolytics are commonly utilized although studies in non-cystic fibrosis related disease have not found significant benefit with therapy. Acetylcysteine, Pulmozyme®, and hypertonic saline are the most frequently used agents. Mechanical therapy to aid in secretion clearance is utilized as well. Some techniques are as simple as increasing exercise to increase expiratory flow rates, or patient education for forced exhalation or coughing technique. Chest physiotherapy and postural drainage may also benefit the patient and are inexpensive modalities, however may be limited by patient discomfort. Positive expiratory pressure with or without oscillation is also readily available (acapella or flutter device). External high frequency chest wall compression (ie. VEST®) therapy is also available, however is more expensive, and typically requires preauthorization by most insurance providers. It is tolerated better than standard chest physiotherapy however.
Antibiotics are used for the treatment of acute exacerbations and prevention of exacerbations. Sputum cultures should be obtained prior to antibiotic therapy, and will help with antibiotic choices. Gram negative rods are frequent colonizers of the airways in bronchiectasis. Pseudomonas aeruginosa is associated with more extensive disease and reduced quality of life. Macrolide antibiotics are frequently used to reduce the number of exacerbations and this has been shown in multiple trials. The mechanism of action is not fully understood however. Other antibiotics have been used for daily suppressive therapy, including ciprofloxacin, amoxicillin, and doxycycline. Aerosolized antibiotics such as tobramycin, gentamicin, and aztreonam, have been studied most intensely in patients with cystic fibrosis and Pseudomonas infection. Recently there are additional trials being performed in patients with non-cystic fibrosis related disease, and with other Gram negative chronic bacterial infections. Patients with chronic airway colonization and a history of exacerbations are often encouraged to participate in these studies to help advance our knowledge and discover improved therapies against bronchiectasis.
In the most severe cases, surgical resection may be of benefit to remove chronically infected tissue. This is usually successful if the patient has disease localized to one area, amenable to resection. For those with the most extensive disease, and severe airflow obstruction, lung transplantation may be an option.
A patient with bronchiectasis will often have daily symptoms, and associated morbidity. Early diagnosis of the disease can help prevent further decompensation and progressive bronchial damage. Aggressive therapies can slow the progression of the disease, and provide improvement in the quality of life. Therapies can range from inexpensive simple exercises to a complex, comprehensive chest physiotherapy regimen. Pulmonary consultation is recommended to help these patient achieve their goals with therapy. Monitoring the disease symptoms, radiographic changes, and pulmonary function studies will help guide the course of therapy, and lead to improvement in the quality of life in our patients.
1 Prevalence and economic burden of bronchiectasis [obstructive airways disease]. Weycker D, Edelsberg J, Oster G, Tino G. Clin Pulm Med 2005, 12:205.
2 Human neutrophil peptides and phagocytic deficiency in bronchiectatic lungs. Voglis S, Quinn K, Liu M, et al. Am J Respir Crit Care Med. 2009; 180(2): 159.
3 Shed syndecan-1 restricts neutrophil elastase from alpha-1 antitrypsin in neutrophillic airway inflammation. Chan SC, Leung VO, Ip MS, Shum DK. Am J Respir Cell Mol Biol. 2009; 41(5): 620.
4 Bronchiectasis in a diverse US population: effects of ethnicity on etiology and sputum culture. McShane PJ, Naureckas ET, and Strek ME. Chest. 2012; 142(1): 159.
5 Clinical manifestations of cystic fibrosis among patients with diagnosis in adulthood. Gilljam M, Ellis L, Corey M, Zielenski J, et al. Chest. 2004; 126(4): 1215.
Dr. Kevin De Boer, DO, FCCP, FACOI graduated from the University of Medicine and Dentistry of New Jersey – School of Osteopathic Medicine (UMDNJ-SOM) He completed residency and fellowship training at UMDNJ-SOM and is board certified in Internal Medicine, Pulmonary, and Critical Care Medicine. Dr. DeBoer is currently the Pulmonary Chair for the American Osteopathic Board of Internal Medicine, and is the Department Chair for Pulmonary Medicine for Florida Hospital (Orlando campuses). He works with Central Florida Pulmonary Group and has interests in idiopathic pulmonary fibrosis, interstitial lung disease and non-cystic fibrosis related bronchiectasis. He is the Clinical Director for Research at the Central Florida Pulmonary Group. Many clinical trials are currently available in these areas. For further information or consultation please visit www.cfpulmonary.com or contact Dr. De Boer at (407) 841-1100
By Kevin DeBoer, DO