Disease, Illness and Condition Library


    Bronchiectasis

    Bronchietasis is an irreversible widening (dilation) of portions
    of the airways (bronchi) resulting from damage to the
    bronchial wall.

    Bronchietasis can result from several conditions that injure the bronchial wall
    directly or indirectly by interfering with its normal defenses against potentially
    harmful substances. The most common cause is severe respiratory infections.
    Immune deficiency disorders, hereditary disorders (such as cystic fibrosis, in
    which abnormal mucus impairs the ability of the cilia to clear the bronchi of
    organisms that cause infections), and mechanical factors (such as bronchial
    obstruction caused by an inhaled object, a lung tumor, or other disorders) may
    predispose a person to infections that lead to bronchietasis. A small number
    of cases probably result from inhaling toxic substances that injure the bronchi,
    such as noxious fumes, gases, smoke (including tobacco smoke), and injurious
    dust (silica, coal dust).

    In broncheictasis, areas of the bronchial wall are destroyed and become
    chronically inflamed, ciliated cells are damaged or destroyed, and secretions
    (mucus) accumulate. Also, the bronchial wall becomes less elastic – the
    affected airways become wider and flabby and may develop outpouchings or sacs
    that resemble tiny balloons. The increased mucus production promotes
    the growth of bacteria, often obstructs the bronchi, and leads to pooling of
    infected secretions and further damage to the bronchial wall. The inflammation
    and infection can extend to the small air sacs of the lungs (alveoli) and produce
    pneumonia, scarring, and a loss of functioning lung tissue.

    In severe cases, scarring and a loss of blood vessels in the lung can ultimately
    strain the right side of the heart as the heart tries to pump blood through the
    altered vessels. Also, inflammation and an increased number of blood vessels
    in the bronchial wall (which are fragile) can result in a person coughing up
    blood. Blockage of the damaged airways can lead to abnormally low levels
    of oxygen in the blood.

    Bronchietasis may affect many areas of the lung, or it may appear in only
    one or two areas. Typically, bronchiectasis causes widening of medium sized
    bronchi, but often smaller bronchi become scarred and destroyed.
    Occasionally, a form of bronchiectasis affecting larger bronchi occurs in allergic
    bronchopulmonary aspergillosis, a condition caused by an allergic response to
    the Aspergillus fungus.

    Symptoms

    Bronchiectasis can develop at any age; often, the process begins in early
    childhood. However, symptoms may not emerge until much later. In most
    people, symptoms begin slowly, usually after a respiratory infection, and tend
    to worsen over the years. Most people develop a chronic cough that produces
    sputum; the amount and type of sputum depend on how extensive the disease
    is and whether there is and whether there is a complicating infection. Often, the
    person has coughing spells only early in the morning and late in the day.
    Coughing up blood is common and may be the first or only symptom.

    Recurrent fever or chest pain, with or without frequent bouts of pneumonia,
    may also occur. People with widespread bronchiectasis may develop wheezing
    or shortness of breath; they may also have chronic bronchitis, emphysema, or
    asthma. Very severe cases of bronchiectasis, which occur more commonly in
    underdeveloped countries and in people who have advanced cystic fibrosis,
    may impair breathing and the lung’s ability to oxygenate the blood and free the
    body of carbon dioxide, a condition called respiratory failure. Very severe
    bronchiectasis may also strain the right side of the heart and lead to
    cor pulmonale.

    Diagnosis

    Doctors may suspect bronchiectasis because of a person’s symptoms or the
    presence of a condition thought to cause bronchietasis. Tests are performed
    to confirm the diagnosis and evaluate the extent and location of the disease.
    Chest x-rays can often detect the lung changes caused by bronchiectasis;
    however, occasionally, results are normal. Computed tomography (CT) is
    usually the most sensitive test to identify and confirm the diagnosis and to
    determine the extent and severity of the disease; these are important factors
    when surgical treatment is being considered.

    After bronchiectasis is diagnosed, tests are often done to check for diseases
    that may be contributing to it, if they were not previously identified. Such tests
    may include measuring the immunoglobulin levels in blood, testing for HIV
    infection and other immune system disorders, measuring the salt levels in
    sweat (which are abnormal in those suffering from cystic fibrosis), and
    examining nasal bronchial, or sperm specimens with a special microscope
    and other tests to determine if the cilia are structurally or functionally defective.
    When bronchiectasis is limited to one area – for example, a lung lobe or
    segment – doctors may perform bronchoscopy to determine whether an
    inhaled foreign object or lung tumor is the cause. Other tests may be
    performed to identify underlying diseases, such as allergic
    bronchopulmonary aspergillosis or tuberculosis.

    Genetic testing for cystic fibrosis may be needed when there is a family
    history, repeated respiratory infections, or other suspicious findings in a
    child or young adult, even when other typical features of cystic fibrosis
    are not present.

    Prevention

    Early detection and treatment of conditions that tend to cause bronchiectasis
    may prevent the development of bronchiectasis or reduce its severity.
    More than 50% of broncheictasis in children can be correctly diagnosed
    and promptly treated.

    Childhood immunizations against measles and whooping cough, suitable use
    of antibiotics, and improved living conditions and nutrition have dramatically
    reduced the number of people who develop bronchietasis. Annual influenza
    vaccines, use of the pneumococcal vaccine, and use of appropriate drugs
    early in the course of infections (such as pneumonia and tuberculosis) help
    to prevent bronchiectasis or reduce its severity. Receiving immunoglobulin
    for an immunoglobulin deficiency syndrome may prevent recurring infections.
    In people who have allergic bronchopulmonary aspergillosis, the appropriate
    use of corticosteroids and perhaps the antifungal drug itraconazole may
    reduce bronchial damage that results in bronchiectasis.

    Avoiding toxic fumes, gases, smoke, and injurious dusts also helps prevent
    bronchietasis or reduce its severity. Inhalation of foreign objects into the
    airways by children may be prevented by carefully watching what they put
    in their mouth. Additionally, avoiding over sedation from drugs or alcohol
    and seeking medical attention for neurologic symptoms (such as impaired
    consciousness) or gastrointestinal symptoms (such as difficulty swallowing
    and regurgitation or coughing after eating) may help to prevent aspiration.
    Also, drops of mineral oil or other oils should never be placed in the mouth
    or nose because they can be inhaled into the lungs.

    Treatment and Prognosis

    Treatment of bronchiectasis is aimed at against eradicating infections,
    decreasing the buildup of mucus and inflammation, relieving airway
    obstruction, and reducing complications (such as coughing up of blood,
    low oxygen levels in the blood, respiratory failure, and cor pulmonale).
    Drugs that suppress coughing may worsen the condition and generally
    should not be used.

    Infections are treated with antibiotics, bronchodilators, and physical therapy
    to promote drainage or secretions. Sometimes antibiotics are prescribed for
    a long period to prevent recurring infections, especially in people who have
    cystic fibrosis.

    For inflammation and the buildup of mucus, anti-inflammatory drugs such as
    inhaled corticosteroids and drugs that thin the pus and mucus (mucolytics)
    may also be given, although the effectiveness of mucolytics is uncertain.
    To help drain the mucus, postural drainage and chest percussion are used.

    To detect and treat a bronchial obstruction, bronchoscopy can be used before
    severe damage occurs. Rarely, part of a lung needs to be surgically removed.
    Such surgery usually is an option only if the disease is confined to one lung,
    or preferably to one lung lobe or segment. Surgery may be considered for
    people who have recurrent infections despite treatment or who cough up large
    amounts of blood. Alternatively, a doctor may intentionally block a bleeding
    bronchial vessel by using a procedure called bronchial arterial embolization.

    If a person’s blood oxygen level is low, oxygen therapy may help prevent
    complications such as cor pulmonale. If the person has wheezing or shortness
    of breath, corticosteroids taken with or without bronchodilators often help.
    Respiratory failure, if present, should be treated.

    Lung transplantation can be performed in certain people who have advanced
    bronchiectasis, mostly those who also have advanced cystic fibrosis. Five year
    survival rates as high as 65 to 75% have been reported when a heart-lung or
    double lung transplantation is used. Pulmonary function (as measured by the
    amount of air in the lungs and the rate and amount moving in and out of the
    lungs with each breath) usually improves within 6 months, and the improvement
    may be sustained for at least 5 years.

    Overall prognosis for people with bronchiectasis depends on how well
    infection and other complications are prevented or controlled. Because
    other conditions (such as chronic bronchitis, emphysema, pulmonary
    hypertension, cor pulmonale, or other serious diseases that affect the
    whole body (systmeic diseases) diminish the effectiveness of prevention
    and treatment, people with these conditions tend to have a less optimistic
    prognosis.
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