[8] Bronchiectasis may result from a number of infectious and acquired causes, including measles,[11] pneumonia, tuberculosis, immune system problems, as well as the genetic disorder cystic fibrosis.
[3] Symptoms of bronchiectasis commonly include a cough productive of frequent green or yellow sputum lasting months to years.
[21] Systemic symptoms, including fevers, chills, night sweats, fatigue and weight loss may be seen with bronchiectasis.
[3] The complications of bronchiectasis include serious health conditions, such as respiratory failure and atelectasis: collapse or closure of a lung.
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.
[38] 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.
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.
[41] Allergic bronchopulmonary aspergillosis (ABPA) is an inflammatory disease caused by hypersensitivity to the fungus Aspergillus fumigatus.
[39] Bronchiectasis could be caused by: inhalation of ammonia and other toxic gases,[49] chronic pulmonary aspiration of stomach acid from esophageal reflux,[50] or a hiatal hernia.
[55] Tracheobronchomegaly, or Mournier-Kuhn syndrome is a rare condition characterized by significant tracheobronchial dilation and recurrent lower respiratory tract infections.
[56] Individuals with alpha 1-antitrypsin deficiency have been found to be particularly susceptible to bronchiectasis, due to the loss of inhibition to enzyme elastase which cleaves elastin.
[21][3] This triggers a host immune response from neutrophils (elastases), reactive oxygen species, and inflammatory cytokines that results in progressive destruction of normal lung architecture.
[34] Traction bronchiectasis characteristically affects peripheral bronchi (which lack cartilage support) in areas of end-stage fibrosis.
[62] 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.
[22] 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.
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.
[69] The next steps include controlling infections and bronchial secretions, relieving airway obstructions, removing affected portions of lung by surgery, and preventing complications.
[73] The active cycle of breathing technique (ACBT), which can be employed with or without a flutter device, is beneficial in treating those with bronchiectasis.
[3] Mannitol is a hyperosmolar agent that is thought to hydrate airway secretions, however, clinical trials with it have not demonstrated efficacy.
[39] Three large multicenter, randomized trials have shown reduced rates of exacerbations and improved cough and dyspnea with use of macrolide therapy.
[64] The impact of adverse effects of macrolides such as gastrointestinal symptoms, hepatotoxicity, and increased antimicrobial resistance needs ongoing review and study.
[17] Inhaled corticosteroid therapy can reduce sputum production and decrease airway constriction over a period of time, helping prevent progression of bronchiectasis.
[76] Antibiotics are used in bronchiectasis to eradicate P. aeruginosa or MRSA, to suppress the burden of chronic bacterial colonization, and to treat exacerbations.
[80] 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.
The goals are conservative, aiming to control specific disease manifestations rather than cure or eliminate all areas of bronchiectasis.
[81] 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.
[3] In a Medicare cohort study in the United States, consisting of adults 65 years and older, the prevalence of bronchiectasis was 701 per 100,000 persons.
[85] A similar prevalence rate of bronchiectasis has been reported in other countries including China, Germany, the United Kingdom, Spain and Singapore.
[89] 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.