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Figure A shows a cross-section of the lungs with normal airways and widened airways. Figure B shows a cross-section of a normal airway. Figure C shows a cross-section of an airway with bronchiectasis.
Bronchiectasis is a disease state defined by localized, irreversible dilation of part of the bronchial tree caused by destruction of the muscle and elastic tissue. It is classified as an obstructive lung disease, along with emphysema, bronchitis, asthma, and cystic fibrosis.
Involved bronchi are dilated, inflamed, and easily collapsible, resulting in airway obstruction and impaired clearance of secretions. Bronchiectasis may result from a variety of infective and acquired causes, including severe and recurrent pneumonia, tuberculosis, and cystic fibrosis.
Signs and symptoms
Some people with bronchiectasis may produce frequent green/yellow sputum (up to 240ml (8 oz) daily). Bronchiectasis may also present with hemoptysis in the absence of sputum, called "dry bronchiectasis". Sputum production may also occur without coloration. People with bronchiectasis may have bad breath indicative of active infection. Frequent bronchial infections and breathlessness are two possible indicators of bronchiectasis.
Tuberculosis, pneumonia, inhaled foreign bodies, allergic bronchopulmonary aspergillosis and bronchiol tumours are the major acquired causes of Bronchiectasis. Infective causes associated with Bronchiectasis include infections caused by the Staphylococcus, Klebsiella, or Bordetella pertussis, the causative agent of whooping cough.
Aspiration of ammonia and other toxic gases, pulmonary aspiration, alcoholism, heroin (drug use), various allergies all appear to be linked to the development of Bronchiectasis,[source needs translation]
Various immunological and lifestyle factors have also been linked to the development of bronchiectasis:
- Childhood Acquired Immune Deficiency Syndrome (AIDS), which predisposes patients to a variety of pulmonary ailments, such as pneumonia and other opportunistic infections.
- Inflammatory bowel disease, especially ulcerative colitis. It can occur in Crohn's disease as well, but does so less frequently. Bronchiectasis in this situation usually stems from various allergic responses to inhaled fungal spores. A Hiatal hernia can cause Bronchiectasis when the stomach acid that is aspirated into the lungs causes tissue damage.
- Rheumatoid arthritis sufferers who smoke, who appear to have a tenfold increased prevalence of the disease. Still, it is unclear as to whether or not cigarette smoke is a specific primary cause of bronchiectasis.
No cause is identified in up to 50% of non-cystic-fibrosis related bronchiecstasis.
Bronchiectasis may result from congenital infections that affect cilia motility or ion transport. Kartagener syndrome is one such disorder of cilia motility linked to the development of bronchiectasis,. A common cause is cystic fibrosis, which affects chloride ion transport, in which a small number of patients develop severe localized bronchiectasis. Young's syndrome, which is clinically similar to cystic fibrosis, is thought to significantly contribute to the development of bronchiectasis. This is due to the occurrence of chronic infections of the sinuses and bronchiole tree.
Other less-common congenital causes include primary immunodeficiencies, due to the weakened or nonexistent immune system response to severe, recurrent infections that commonly affect the lung. Several other congenital disorders can also lead to bronchiectasis, including Williams-Campbell syndrome and Marfan syndrome.
Bronchiectasis is a result of chronic inflammation compounded by an inability to clear mucoid secretions. This can be a result of genetic conditions resulting in a failure to clear sputum (Primary ciliary dyskinesia), or resulting in more viscous sputum (cystic fibrosis), or the result of chronic or severe infections. Inflammation results in progressive destruction of the normal lung architecture, in particular the elastic fibres of bronchi.
Bronchiectasis may be diagnosed clinically or on review of imaging. The British Thoracic Society recommends all non cystic-fibrosis-related bronchiectasis be confirmed by CT. CT may reveal tree-in-bud abnormalities, dilated bronchi, and cysts with defined borders.
In order to prevent bronchiectasis, children should be immunized against measles, pertussis and other acute respiratory infections of childhood. While smoking has not been found to be a direct cause of bronchiectasis, it is certainly an irritant that all patients should avoid in order to prevent 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 pneumotherapy. Aggressively treating bronchial infections with antibiotics to prevent the destructive cycle of infection, damage to bronchial tubes, 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.
Treatment of bronchiectasis includes controlling infections and bronchial secretions, relieving airway obstructions, removal of affected portions of lung by surgical removal or artery embolization and preventing complications. This includes the prolonged usage of antibiotics to prevent detrimental infections, as well as eliminating accumulated fluid with postural drainage and chest physiotherapy. Postural drainage techniques, aided by physiotherapists, are an important mainstay of treatment.
Surgery may also be used to treat localized bronchiectasis, removing obstructions that could cause progression of the disease.
Inhaled steroid therapy that is consistently adhered to can reduce sputum production and decrease airway constriction over a period of time, and help prevent progression of bronchiectasis. This is not recommended for routine use in children. One commonly used therapy is beclometasone dipropionate, which is also used in asthma treatment. Use of inhalers such as albuterol (salbutamol), fluticasone (Flovent/Flixotide) and ipratropium (Atrovent) may help reduce likelihood of infection by clearing the airways and decreasing inflammation.
Although not approved for use in any country, Mannitol dry inhalation powder, under the name Bronchitol, has been granted orphan drug status by the FDA for use in patients with bronchiectasis and with cystic fibrosis.
Additionally, the use of ACBT (Active Cycle Breathing Techniques) can be useful in the clearance of sputum. These techniques encourage relaxed, diaphragmatic breathing, greater expansion (via collateral inflation) of otherwise consolidated areas of the lungs, and help in mucociliary clearance (MCC). A useful adjunct to these cycles are manual techniques, wherein the healthcare professional uses percussion, vibrations, and shaking, to dislodge sputum from the chest walls, enabling the patient to expectorate more easily.
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Rene Laënnec, the man who invented the stethoscope, used his invention to first discover bronchiectasis in 1819. The disease was researched in greater detail by Sir William Osler in the late 1800s; it is suspected that Osler actually died of complications from undiagnosed bronchiectasis.
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