The disease affects between 1 per 1000 and 1 per 250,000 adults. The disease is more common in women and increases as people age. It became less common since the 1950s with the introduction of antibiotics. It is more common among certain ethnic groups such as indigenous people. It was first described by René Laennec in 1819. The economic costs in the United States are estimated at $630 million per year.
Signs and symptomsEdit
The typical symptoms of bronchiectasis are shown. Also, the change in bronchi under bronchiectasis are illustrated.
Symptoms of bronchiectasis commonly include a cough productive of frequent green or yellow sputum lasting months to years. Other common symptoms include difficulty breathing, wheezing (a whistling sound when you breathe), and chest pain. Bronchiectasis may also present with coughing up blood in the absence of sputum, which has been called "dry bronchiectasis."
People often report frequent bouts of "bronchitis" requiring therapy with repeated courses of antibiotics. People with bronchiectasis may have bad breath from active infection. On examination, crepitations and expiratory rhonchi may be heard with auscultation. Nail clubbing is a rare symptom.
The complications of bronchiectasis include serious health conditions, such as respiratory failure and atelectasis:
collapse or closure of a lung. Respiratory failure occurs when not enough oxygen passes from the lungs into the blood. Atelectasis occur when one or more segments of the lungs collapse or do not inflate properly. Other pulmonary complications include lung abscess and empyema. Cardiovascular complications include cor pulmonale, in which there is enlargement and failure of the right side of the heart as a result of disease of the lungs.
There are many causes that can induce or contribute to the development of bronchiectasis. The frequency of these different causes varies with geographic location.Cystic fibrosis is identified as a cause in up to half of cases. Bronchiectasis without CF is known as non-CF bronchiectasis. Historically, about half of all case of non-CF bronchiectasis were found to be idiopathic, or without a known cause. However, more recent studies with a more thorough diagnostic work-up have found an etiology in 60 to 90% of patients.
An airway obstruction can be caused by either an intraluminal mass such as a tumor or a foreign body. The presence of an airway obstruction leads to a cycle of inflammation. It is important to identify the presence of an obstruction because surgical resection is often curative if obstruction is the cause. In adults, foreign body aspiration is often associated with an altered state of consciousness. The foreign body is often unchewed food, or part of a tooth or crown. Bronchiectasis that results from foreign body aspiration generally occurs in the right lung in the lower lobe or posterior segments of the upper lobe.
Impairments in host defenses that lead to bronchiectasis may be congenital, such as with primary ciliary dyskinesia, or acquired, such as with the prolonged use of immunosuppressive drugs. Additionally, these impairments may be localized to the lungs, or systemic throughout the body. In these states of immunodeficiency, there is a weakened or absent immune system response to severe infections that repeatedly affect the lung and eventually result in bronchial wall injury.HIV/AIDS is an example of an acquired immunodeficiency that can lead to the development of bronchiectasis.
Allergic bronchopulmonary aspergillosis (ABPA) is an inflammatory disease caused by hypersensitivity to the fungus Aspergillus fumigatus. It is suspected in patients with a long history of asthma and symptoms of bronchiectasis such as a productive, mucopurulent cough. Imaging often shows peripheral and central airway bronchiectasis, which is unusual in patients with bronchiectasis caused by other disorders.
A causal role for tobacco smoke in bronchiectasis has not been demonstrated. Nonetheless, tobacco smoking can worsen pulmonary function and accelerate the progression of disease that is already present.
"Vicious cycle" theory of the pathogenesis of bronchiectasis.
The development of bronchiectasis requires two factors: an infectious insult and impaired drainage, obstruction, or a defect in host defense. This triggers a host immune response from neutrophils (elastases), reactive oxygen species, and inflammatory cytokines that results in progressive destruction of normal lung architecture. In particular, the elastic fibers of bronchi are affected. The result is permanent abnormal dilation and destruction of the major bronchi and bronchiole walls.
The "vicious cycle" theory is the generally accepted explanation for the pathogenesis of bronchiectasis. In this model, a predisposed individual develops an excessive inflammatory response to pulmonary infection or tissue injury. The inflammation that results is partially responsible for the structural damage to the airways. The structural abnormalities allow for the stasis of mucus, which favors continued chronic infection and the persistence of the vicious cycle.
Endobronchial tuberculosis commonly leads to bronchiectasis, either from bronchial stenosis or secondary traction from fibrosis. Traction bronchiectasis characteristically affects peripheral bronchi (which lack cartilage support) in areas of end-stage fibrosis.
CT scan of the lungs showing findings diagnostic of bronchiectasis. White and black arrows point to dilated bronchi characteristic of the disease.
The goals of a diagnostic evaluation for bronchiectasis are radiographic confirmation of the diagnosis, identification of potential treatable causes, and functional assessment of the patient. A comprehensive evaluation consists of radiographic imaging, laboratory testing, and lung function testing.
A chest x-ray is abnormal in most patients with bronchiectasis. Computed tomography is recommended to confirm the diagnosis and is also used to describe the distribution and grade the severity of the disease. Radiographic findings include airway dilation, bronchial wall thickening, and atelectasis. There are three types bronchiectasis that can be seen on CT scan, namely cylindrical, varicose, and cystic bronchiectasis.
Bronchiectasis primarily in the middle lobe of the right lung.
Bronchiectasis secondary to a large carcinoid tumor (not shown) that was completely obstructing the bronchus proximally. Dilation of the airways is present.
In preventing bronchiectasis, it is necessary to prevent the lung infections and lung damage that can cause it. Children should be immunized against measles, pertussis, pneumonia, and other acute respiratory infections of childhood. Additionally, parents should stay alert to keep children from inhaling objects such as pieces of food or small toys that may get stuck in small airways.Smoking and other toxic fumes and gases should be avoided by all patients with bronchiectasis to decrease the development of infections (such as bronchitis) and further complications.
Treatments to slow down the progression of this chronic disease include keeping bronchial airways clear and secretions weakened through various forms of airway clearance. Aggressively treating bronchial infections with antibiotics to prevent the destructive cycle of infection, damage to bronchi and bronchioles, and more infection is also standard treatment. Regular vaccination against pneumonia, influenza, and pertussis are generally advised. A healthy body mass index and regular doctor visits may have beneficial effects on the prevention of progressing bronchiectasis. The presence of hypoxemia, hypercapnia, dyspnea level and radiographic extent can greatly affect the mortality rate from this disease.
A comprehensive approach to the management of bronchiectasis is recommended. It is important to establish whether an underlying modifiable cause, such as immunoglobulin deficiency or alpha-1 antitrypsin deficiency is present. The next steps include controlling infections and bronchial secretions, relieving airway obstructions, removing affected portions of lung by surgery, and preventing complications.
Antibiotics are used in bronchiectasis to eradicate P. aeruginosa or MRSA, to suppress the burden of chronic bacterial colonization, and to treat exacerbations. The use of daily oral non-macrolide antibiotic treatment has been studied in small case series, but not in randomized trials. The role of inhaled antibiotics in non-CF bronchiectasis has recently evolved with two society guidelines and a systematic review suggesting a therapeutic trial of inhaled antibiotics in patients with three or more exacerbations per year and P. aeruginosa in their sputum. Options for inhaled antibiotics include aerosolized tobramycin, inhaled ciprofloxacin, aerosolized aztreonam, and aerosolized colistin.
Some clinical trials have shown a benefit with inhaled bronchodilators in certain people with bronchiectasis. In people with demonstrated bronchodilator reversibility on spirometry, the use of inhaled bronchodilators resulted in improved dyspnea, cough, and quality of life without any increase in adverse events. However, overall there is a lack of data to recommend use of bronchodilators in all patients with bronchiectasis.
The primary role of surgery in the management of bronchiectasis is in localized disease to remove segments of the lung or to control massive hemoptysis. Additionally, surgery is used to remove an airway obstruction that is contributing to bronchiectasis. The goals are conservative, aiming to control specific disease manifestations rather than cure or eliminate all areas of bronchiectasis. Surgical case series have shown low operative mortality rate (less than 2%) and improvement of symptoms in the majority of patients selected to receive surgery. However, no randomized clinical trials have been performed evaluating the efficacy of surgery in bronchiectasis.
Results from a phase 2 clinical trial have recently been published. In a placebo-controlled, double-blind study conducted in 256 patients worldwide, patients who received Brensocatib reported prolonged time to the first exacerbation and also reduced rate of yearly exacerbation.
The disease affects between 1 per 1000 and 1 per 250,000 adults. The disease is more common in women and increases as people age. It became less common since the 1950s, with the introduction of antibiotics. It is more common among certain ethnic groups such as indigenous people.
An estimated 350,000 to 500,000 adults have bronchiectasis in the United States. The disease is more common in women and in elderly individuals over the age of 65. Specifically, children of the indigenous populations of Australia, Alaska, Canada and New Zealand have significantly higher rates than other populations. Overall, a shortage of data exists concerning the epidemiology of bronchiectasis in Asia, Africa, and South America.
The exact rates of bronchiectasis are often unclear as the symptoms are variable. Rates of disease appeared to have increased in the United States between 2000 and 2007.
The disease was researched in greater detail by Sir William Osler, one of the four founding professors of Johns Hopkins Hospital, in the late 1800s. It is suspected that Osler himself died of complications from undiagnosed bronchiectasis. His biographies mention that he had frequent severe chest infections for many years.
The term "bronchiectasis" comes from the Greek words bronkhia (meaning "airway") and ektasis (meaning "widening").
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