Bronchiectasis is a chronic lung disease defined by irreversible dilation of the bronchi. It develops from an initial event that impairs mucociliary clearance, allowing bacteria to colonize and further damage the lungs. The hallmark symptoms are a chronic cough and sputum production lasting months to years. Risk factors include cystic fibrosis, infections, immunodeficiencies, and exposures to toxic substances. Management involves antibiotics, airway clearance techniques, and supplemental oxygen. Severe cases may require surgery.

1. BRONCHIECTASIS
THANUJA ELEENA MATHEW

2. INTRODUCTION
• Bronchiectasis is a chronic lung disease, defined pathologically as irreversible
dilatation of the bronchi. The clinical course of the disease is chronic and
progressive and in most cases, causes lung damage over many years. There is
usually an initial event, which causes impairment of mucociliary clearance of
the bronchial tree.
The respiratory tract becomes colonized by bacteria that inhibit the ciliary
function and promote further lung damage. The hallmark of bronchiectasis, is a
chronic cough with mucopurulent or purulent sputum, lasting for months to
years and may progress to chronic respiratory failure. In this comprehensive
review, we discuss the etiology, pathogenesis, clinical presentation, appropriate
investigations and management of bronchiectasis.

3. DEFINITION
• Bronchiectasis is a condition in which the bronchial tubes in
the lung become damaged from inflammation or other causes
and the smooth muscles of the bronchial tubes are destroyed.
In addition, elasticity of the bronchi is often lost.
Bronchiectasis may be acquired or have a genetic origin.
Many clinicians consider bronchiectasis to be a form
of chronic obstructive pulmonary disease (COPD); it
includes chronic bronchitis and emphysema

4. INCIDENCE
• The prevalence and incidence of bronchiectasis are not known accurately. Prevalence
has been estimated in people aged 18–34 years, rising to 272 per 100 000 in over those
over 75 years of age in the USA in 2005.
• To put the prevalence in context, these estimates suggest there may be 1 patient with
bronchiectasis for every 20 patients with chronic obstructive pulmonary disease
(COPD) in Western countries.
• Incidence increased each year between 2014 and 2016, from 18/100,000 person-years at
risk, to 32/100,000 person-years at risk.
• Prevalence was higher in women than in men.
• Prevalence was higher in the older age groups (>60 years).
• Bronchiectasis can present at any age but increases with age and the highest prevalence
is in older women. Up to 70% of cases may be in women.

5. RISK FACTORS
• individuals with alpha-1 anti-proteinase (alpha-1 antitrypsin) deficiency or an
embryological defect termed immotile cilia syndrome.
• People with cystic fibrosis
• Children that develop lung infections with lung tissue destruction are risk for
bronchiectasis to develop later in life.
• People that have recurrent lung infections, aspirate foreign bodies, have had a history
of tuberculosis or inflammatory bowel disease.
• People that abuse drugs and alcohol.
• Individuals that are exposed to toxic gases or any substances that damage lung tissue

6. TYPES
• Cylindrical bronchiectasis, the mildest form of bronchiectasis that shows
the loss of normal airway tapering.
• Saccular or varicose bronchiectasis shows further distortion of the
airway wall along with more mucous and sputum production by the
individual; some of the bronchi may appear to be in a beaded form.
• Cystic bronchiectasis: The most severe form of bronchiectasis and the
least common form is cystic bronchiectasis. This form has large air spaces
and a honeycombed appearance in CT scan studies and usually has thicker
walls than the blebs seen with emphysema. Some people have more than
one type in their lungs.

7. ETIOLOGY
• Cystic fibrosis
• Low BMI
• Toxic fumes, gases, smoke, and other
harmful substances
• Allergic Bronchopulmonary
Aspergillosis (ABPA)
• Immunodeficiencies
• Connective tissue disease
• Exposure to chemical irritants
• Retained foreign object
• Primary ciliary dyskinesia
• Alpha 1- anti trypsin deficiency
• Rheumatoid arthritis
• Alcohol or drug abuse
• Inflammatory bowel disorder
• Childhood infections such as
pneumonia, tuberculosis, measles,
whooping cough, adenovirus,
and Mycoplasma pneumoniae

9. CLINICAL MANIFESTATION
• Coughing up lots of sputum
• Foul smelling mucus
• Tiredness and poor concentration
• Wheeziness
• Some people became breathless,
particularly when exercising or exerting
themselves.
• Patient may cough up some blood from
an inflamed airway [haemoptysis]
• A constant runny nose
• Chest pain and join pain
• Recurring chest infections
• Abnormal chest sounds
• Weakness
• Weight loss
• Fatigue
• Clubbing of fingers [the flesh under
fingernails and toenails gets thicker]
• Some people with bronchiectasis also
have chronic [persistent] sinusitis

10. DIAGNOSTIC EVALUATION
• History collection
• Physical examination
• Chest CT Scan
• Chest X Ray
• Other Tests
• Blood tests.
• Lung function tests.
• Bronchoscopy
• Pulmonary function test

11. MANAGEMENT
• Antibiotics – based on regular assessment of airway microbiology
• Bronchodilators – bronchiectasis is not always associated with reversible airway dysfunction
and determination of bronchodilator response in the pulmonary function laboratory should
precede institution of long-term bronchodilator therapy.
• Corticosteroids – While some conditions resulting in bronchiectasis may respond to steroid
treatment including even oral steroid therapy care should be taken in children with
bronchiectasis to ensure that regular assessment of the benefit of such therapy is balanced
against the potential side effect of prolonged therapy in the paediatric patient.
• Mucus thinner agents – Improved airway clearance may be achieved with the regular inhalation
of mucolytic agents such as hypertonic saline (6 or 7%) or mannitol. The mucolytic therapy used
as a mucolytic agent in CF has not been shown to be generally effective in bronchiectasis caused
by conditions other than CF and, given its significant cost, should not be used.
• Macrolide therapy – Several studies have shown benefit to the use of azithromycin in retarding
the progression of bronchiectasis from conditions as Pseudomonas Aeruginosa positive CF
patients and COPD in adults by preserving lung function and reducing the number of acute
exacerbations. Therapy is aimed at a direct anti-inflammatory action rather than an antimicrobial
action and thus dosing is often taken as single dose daily or three times a week

12. • Management of acute exacerbations
• During an exacerbation, there is often a proliferation of bacterial pathogens and
increased airway and systemic inflammation. Treatment with antibiotics has been
shown to reduce bacterial burden as well as inflammation. Deciding when a patient
has an acute exacerbation is based on symptomatic and physiologic changes, rather
than any specific lab investigation. An acute bacterial infection is often preceded by an
increase in sputum volume and sputum purulence. There may also be additional
symptoms including malaise, dyspnoea, pleuritic chest pain and/or haemoptysis.
Systemic symptoms such as fevers, sweats and rigors may be present, but many
patients will have an exacerbation without those symptoms. Viral infection can also
trigger exacerbations.

13. • Antibiotic Therapy
• In patients with beta-lactamase producing organisms, empiric
treatment with amoxilcillin or amoxicillin and clavulinic acid is
appropriate. For patients allergic to penicillin, doxycycline is an
appropriate alternative. For patients known to be colonized with
Pseudomonas aeruginosa, ciprofloxacin is an appropriate agent.
Patients who have had MRSA previously isolated can be treated with a
combination of rifampicin and fusidic acid. If previous sputum isolates
are not available, then empiric therapy targeting Haemophilus
influenzae is appropriate.

14. • Indications for Hospitalisation
• If a patient requires intravenous antibiotics, hospitalisation is indicated. For patients
who have Pseudomonas aeruginosa resistant to ciprofloxacin, intravenous therapy is
indicated. Other indications for hospitalisation include the presence of respiratory
failure with a respiratory rate >/= 25/min, hypotension, fever >38C, hypoxic
respiratory failure with SpO2 </= 92% or failure to improve after a course of oral
antibiotics. It is important that patients admitted to hospital are also treated with
appropriate airway clearance techniques.
• Supplemental Oxygen
• There is little evidence to support or oppose the long-term use of oxygen therapy to
reduce mortality in patients with chronic respiratory conditions other than COPD
Supplemental oxygen therapy may be used if there is evidence of hypoxic respiratory
failure (SpO2 < 90% or PaO2 < 65mmHg). While this may improve oxygenation, it will
not necessarily have any impact on dyspnoea. If supplemental oxygen is used, it is
appropriate to maintain a saturation of >92%.The addition of oxygen therapy to a
patient’s management plan should be determined by a comprehensive clinical
assessment, not just the requirement of an increase in PaO2.

15. SURGICAL MANAGEMENT
•Lung volume reduction surgery, during which small
wedges of damaged lung tissues are removed, may be
recommended for some patients with severe cases of
bronchiectasis.
•Lung transplantations: in very severe cases lung
transplantation may be an option for some patients.

16. PREVENTION
• Bronchiectasis can be prevented by effectively preventing the lung
infections and lung damage that can cause the condition. Vaccines
for conditions such as measles and whooping cough help prevent
infections that may lead to bronchiectasis. Avoiding pollution and
toxic gases, smoke, and other harmful substances can also help
protect the lungs. Early and effective treatment of lung infections
may also help preserve lung function reducing the risk of
bronchiectasis. Treating the underlying cause of bronchiectasis can
delay or prevent the progression of the condition.

17. CONCLUSION
• Bronchiectasis is still, one of the frequently seen chronic lung diseases, that can affect
the life quality and expectancy of the affected person. Multiple conditions are
associated with the development of bronchiectasis, but all require both an infectious
insult plus impairment of drainage, airway obstruction and/or a defect in host defence.
Many attempts have been made to treat this disease, but none of the treatment options
altered the natural course of the disease. Treatment of bronchiectasis is aimed at
controlling infection and improving bronchial hygiene. Surgical extirpation of affected
areas may be useful in selected patients.

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