From Wikipedia, the free encyclopedia
Bronchiectasis is a disease where there is permanent enlargement of parts of the
airways of the
lung.
[1] Symptoms typically include a chronic cough with
sputum production.
[2] Other symptoms include
shortness of breath,
coughing up blood and chest pain.
[1] Wheezing and
nail clubbing may also occur. Those with the disease often get frequent
lung infections.
[1]
Bronchiectasis may result from a number of
infective and acquired causes, including pneumonia,
tuberculosis,
immune system problems, and
cystic fibrosis.
[2][3] Cystic fibrosis eventually results in severe bronchiectasis in nearly all cases.
[4]
The cause in 10-50% of those without cystic fibrosis is unknown. The
mechanism of disease is breakdown of the airways due to an excessive
inflammatory response. Involved
bronchi become enlarged and thus less able to clear secretions. These secretions increase the amount of
bacteria in the lungs, result in airway blockage and further breakdown the airways.
[2] It is classified as an
obstructive lung disease, along with
chronic obstructive pulmonary disease and
asthma.
[5] The diagnosis is suspect based on a person's symptoms and confirmed using
computer tomography.
[6] Sputum cultures may be useful to determine treatment in those who have acute worsening
[1] and at least once a year.
[6]
Worsening may occur due to infection and in these cases
antibiotics are recommended. Typical antibiotics used include
amoxicillin and in those who are allergic
erythromycin or
doxycycline.
[1] Antibiotics may also be used to prevent worsening of disease.
[2] Airway clearance techniques, a type of
physiotherapy, are recommended.
[7] Bronchodilators may be useful in some but the evidence is not very good.
[2] The use of
inhaled steroids have not been found to be useful.
[8] Surgery, while commonly done, has not been well studied.
[9] Lung transplantation may be an option in those with very severe disease.
[10] While the disease may cause significant health problems
[2] many other people with the disease do well.
[1]
In the United Kingdom the disease affects about 1 per 1000 adults.
[1] The disease is more common in women and increases as people age. It was first described by
Rene Laennecin 1819. The economic costs in the United States are estimated at $630 million per year.
[2]
Signs and symptoms
Some people with bronchiectasis may produce frequent green/yellow
sputum (up to 240ml (8 oz) daily). Bronchiectasis may also present with
coughing up blood
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.
[3]
Crepitations and expiratory
rhonchi may be heard on auscultation, although
nail clubbing is rare.
[11]
Causes
Bronchiectasis has both
congenital and
acquired causes, with the latter more frequent.
[12]
Acquired causes
Bronchiectasis secondary to a large carcinoid tumor (not shown) that was
completely obstructing the bronchus proximally. The yellowish
discoloration of lung parenchyma reflects obstructive pneumonia.
Tuberculosis, pneumonia, inhaled foreign bodies,
allergic bronchopulmonary aspergillosis and
bronchial tumours are the major acquired causes of Bronchiectasis.
[3][13] Infective causes associated with Bronchiectasis include infections caused by the
Staphylococcus,
Klebsiella, or
Bordetella pertussis, the causative agent of
whooping cough.
[12]
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,
[14][source needs translation]
Various immunological and lifestyle factors have also been linked to the development of bronchiectasis:
No cause is identified in up to 50% of non-cystic-fibrosis related bronchiecstasis.
[11]
Congenital causes
Bronchiectasis may result from congenital infections that affect
cilia motility or
ion transport.
[3] Kartagener syndrome is one such disorder of
cilia motility linked to the development of bronchiectasis,.
[18] A common cause is
cystic fibrosis, which affects
chloride ion transport, in which a small number of patients develop severe localized bronchiectasis.
[19] 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.
[20]
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.
[21] Several other congenital disorders can also lead to bronchiectasis, including
Williams-Campbell syndrome[22] and
Marfan syndrome.
[23]
Patients with
alpha 1-antitrypsin deficiency have been found to be particularly susceptible to bronchiectasis, for unknown reasons.
[24]
Pathophysiology
Bronchiectasis, gross pathology.
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.
[3]
Tuberculosis. Endobronchial tuberculosis commonly leads to bronchiectasis, either from bronchial
stenosis or secondary
traction from fibrosis.
[25]
Diagnosis
Bronchiectasis may be diagnosed clinically or on review of imaging.
[3][26] The British Thoracic Society recommends all non cystic-fibrosis-related bronchiectasis be confirmed by CT.
[11] CT may reveal
tree-in-bud abnormalities, dilated bronchi, and cysts with defined borders.
[3]
Other investigations typically performed at diagnosis include
blood tests,
sputum cultures, and sometimes tests for specific
genetic disorders.
[3]
Prevention
In order to prevent bronchiectasis, children should be immunized against measles,
pertussis,
pneumonia, 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.
[27]
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.
[28]
Treatment
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,
[29] as well as eliminating accumulated fluid with
postural drainage and chest
physiotherapy. Postural drainage techniques, aided by
physiotherapists and respiratory therapists, are an important mainstay of treatment.
[3][11] Airway clearance techniques appears useful.
[7]
Surgery may also be used to treat localized bronchiectasis, removing obstructions that could cause progression of the disease.
[30]
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.
[11] One commonly used therapy is
beclometasone dipropionate.
[31] Although not approved for use in any country,
mannitol dry inhalation powder, has been granted
orphan drug status by the
FDA for use in people with bronchiectasis and with cystic fibrosis.
[32]
Epidemiology
In the United Kingdom the disease affects about 1 per 1000 adults.
[1]
History
René Laennec, the man who invented the
stethoscope, used his invention to first discover bronchiectasis in 1819.
[33] 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.
[34]
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