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Chronic Care Programme
Treatment guidelines
Bronchiectasis
 n/a
Chronic condition
Alternative names
Consultations protocols
Preferred treating provider
Notes
 preferred as indicated by option
 referral protocols apply
Maximum consultations per annum
 Initial consultation
 Follow-up consultation
Tariff codes
Physiotherapy:
Treating doctor:
complex
evaluation /
Physiotherapist
counselling at
first visit only
 Initial consultation
 Follow-up consultation
Physiotherapy:
Treating doctor:
one complete
reassessment of
Physiotherapist
patient’s
condition during
the course of
treatment
 Initial consultation
Option/plan
Provider
GMHPP
Gold Options
G1000, G500
and G200.
Blue Options
B300 and B200.
GMISHPP
General Practitioner
Physician
Paediatrician
Cardiologist
Pulmonologist
Surgeon
Thoracic Surgeon
New Patient
Existing patient
Mild / Stable
Moderate to
Severe /
Unstable
1
3
0183; 0142; 0187; 0108
1
1
New Patient
Existing patient
Mild / Stable
Moderate to
Severe /
Unstable
2
2
New Patient
 Follow-up consultation
1
3
2
Existing patient
4
Mild / Stable
Moderate to
Severe /
Unstable
2
4
Investigations protocols
Type
Provider
Tariff code
Physiotherapy – postural
draininage
Flow volume test:
Inspiration / expiration
Peak expiratory flow only
Maximum investigations per annum
New patient
Existing patient
Stable
Unstable
Controlled Uncontrolled
6
4
6
Physiotherapist
0315
Any preferred
provider
Any preferred
1186
2
1
2
1192
2
1
2
Nebulizations in rooms
Sputum microscopy
Sputum bacteriological
culture
Sputum fungal culture
Sputum mycobacterium
Antibiotic susceptibility test:
per organism
Blood gases: (Panel 1:
Astrup/p02. This panel
includes items 4077, 4078 &
4021)
CXR
ICD 10 coding
provider
Any preferred
provider
Any preferred
provider
Pathologist
1136
1
1
1
3867
2
1
2
3895
2
1
2
Pathologist
Pathologist
Pathologist
3901
3915
3887
2
2
6
1
1
3
2
2
6
Pathologist or as
per treating
doctor
4075
1
0
1
3445
2
1
2
Radiologist
J47., Q33.4
General
Bronchiectasis is a disease that causes localized, irreversible dilatation of part of the bronchial
tree. Involved bronchi are dilated, inflamed, and easily collapsible, resulting in airflow
obstruction and impaired clearance of secretions. Bronchiectasis is associated with a wide range
of disorders, but it usually results from necrotizing bacterial infections, such as infections caused
by the Staphylococcus or Klebsiella species or Bordetella pertussis.[1]
Rene Theophile Hyacinthe Laënnec, the man who invented the stethoscope, used his creation to
first discover bronchiectasis in 1819.[2]. The disease was researched in greater detail by Sir
William Osler in the late 1800s; in fact, it is suspected that Osler actually died of complications
from undiagnosed bronchiectasis[3].

Bronchiectasis is a condition in which an area of the bronchial tubes is permanently and
abnormally widened (dilated), with accompanying infection.

Description. The bronchial tubes are the networks of branching tubes which deliver air to
the tiny sacs of the lungs (alveoli). In bronchiectasis, the diameter of the bronchi is
unusually large. Examination of the walls of the bronchial tubes reveals destruction of the
normal structural elements, with replacement by scar tissue. Pus collects within the
bronchi, and the normal flow of oxygen into the lungs, and carbon dioxide out of the lungs
(air exchange) is impaired. The bronchi show signs of inflammation, with swelling and
invasion by a variety of immune cells. The inflamed areas show signs of increased growth
of blood vessels. The area of the lung which should be served by a diseased bronchial tube
is also prone to inflammation and infection.
Causes
There are both congenital and acquired causes of bronchiectasis. Kartagener syndrome, which
affects the mobility of cilia in the lungs[7], aids in the development of the disease. Another
common genetic cause is cystic fibrosis, in which a small number of patients develop severe
localized bronchiectasis[8]. 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, sinopulmonary infections.[9] Patients with alpha 1-antitrypsin deficiency
have been found to be particularly susceptible to bronchiectasis, for unknown reasons. [10] 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.[11]
Acquired bronchiectasis occurs more frequently, with one of the biggest causes being
tuberculosis. Endobronchial tuberculosis commonly leads to bronchiectasis, either from bronchial
stenosis or secondary traction from fibrosis.[12] An especially common cause of the disease in
children is acquired immune deficiency syndrome, stemming from the human immunodeficiency
virus. This disease predisposes patients to a variety of pulmonary ailments, such as pneumonia
and other opportunistic infection.[13]. Bronchiectasis can sometimes be an unusual complication of
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 fungus spores.[14] Recent evidence has shown an increased risk of
bronchiectasis in patients with rheumatoid arthritis who smoke. One study stated a tenfold
increased prevalence of the disease in this cohort[15]. Still, it is unclear as to whether or not
cigarette smoke is a specific primary cause of bronchiectasis.
Other acquired causes of bronchiectasis involving environmental exposures include respiratory
infections, obstructions, inhalation and aspiration of ammonia and other toxic gases, pulmonary
aspiration, alcoholism, heroin (drug use), and various allergies.[16]







Prior to the widespread use of immunizations, bronchiectasis was often the result of a
serious infection with either measles or whooping cough. Currently, viruses that cause
influenza (flu) or influenza-like syndromes, as well as a number of bacteria may precede
the development of bronchiectasis. Patients who have been infected with tuberculosis or
the virus which causes AIDS (HIV or human immunodeficiency virus) also have an
increased chance of bronchiectasis.
A number of pre-existing conditions may cause an individual to be more susceptible than
normal to infection, with increased risk of bronchiectasis developing.
These conditions include disorders of cilia, and immune disorders.
Cilia are the tiny hairs which usually line the bronchial tubes. Cilia wave constantly,
sweeping the bronchial tubes clean of bacterial or viral invaders, and cleaning away
excess secretions (mucus, sputum) which may be produced by the bronchi. When these
cilia are abnormal or absent at birth, various bacterial or viral invaders may remain in the
respiratory tract, multiply, and cause serious infections.
Immune disorders include decreased production of certain immune chemicals
(immunoglobulins) which usually serve to fight off infection by bacterial or viral invasion.
When these immunoglobulins are not produced in large enough quantity, bacterial and
viral invaders are not effectively killed off, and infection occurs.
Other causes of bronchiectasis include an abnormally blocked (obstructed) airway. This
can be due to tumor growth within the bronchial tube, or due to a child accidentally
inhaling a small object which then blocks off the bronchial tube. People with the disease
called cystic fibrosis (CF) often have their bronchial tubes obstructed by the thick, sticky
mucus which is a hallmark of CF.
Toxic exposures (breathing ammonia, for example) can harm the bronchi, and lead to
bronchiectasis. An extreme allergic response of the immune system to the presence of
certain fungi (especially one called Aspergillus) can also damage the bronchial tubes
enough to result in bronchiectasis.
Signs and symptoms
Pathogenesis
Dilation of the bronchial walls results in airflow obstruction and impaired clearance of secretions
because the dilated areas disrupt normal air pressure in the bronchial tubes, causing sputum to
pool inside the dilated areas instead of being pushed upward[4]. The pooled sputum provides an
environment conducive to the growth of infectious pathogens, and these areas of the lungs are
thus very vulnerable to infection. The more infections that the lungs experience, the more
damaged the lung tissue and alveoli become. When this happens, the bronchial tubes become
more inelastic and dilated, creating a self-perpetuating cycle of further damage to the lungs.
There are three types of brochiectasis, varying by level of severity. Fusiform (cylindrical)
bronchiectasis (the most common type) refers to mildly inflamed bronchi that fail to taper
distally. In varicose bronchiectasis, the bronchial walls appear beaded, because areas of dilation
are mixed with areas of constriction. Saccular (cystic) bronchiectasis is characterized by severe
and irreversible ballooning of the bronchi peripherally, with or without air-fluid levels.[5] Chronic
productive cough is prominent, occurring in up to 90% of patients with bronchiectasis. Sputum is
produced on a daily basis in 76% of patients.[6]

Clinical Signs and Symptoms of bronchiectasis include constant cough and the
production of infected sputum (sputum is a mixture of mucus and pus), which may be
bloody. In some cases, there may be wheezing and shortness of breath. The constant, lowlevel of infection may flare, resulting in increased production of sputum, worsening of the
cough, and fever. The area of the lung served by the affected bronchial tube may become
severely infected, resulting in pneumonia.
Diagnosis
The diagnosis of bronchiectasis is based on the review of clinical history and characteristic
patterns in high-resolution CT scan findings. Such patterns include "tree-in-bud" abnormalities
and cysts with definable borders. In one small study, CT findings of bronchiectasis and multiple
small nodules were reported to have a sensitivity of 80%, specificity of 87%, and accuracy of
80% for the detection of bronchiectasis. Bronchiectasis may also be diagnosed without CT scan
confirmation if clinical history clearly demonstrates frequent, respiratory infections, as well
confirmation of an underlying problem via blood work and sputum culture samples.[17]

Chest x ray may reveal evidence of bronchiectasis, and CT scans are particularly good at
revealing the thick, dilated bronchial walls of bronchiectasis. Sputum will need to be
collected and cultured (grown in a laboratory dish), in order to examine it microscopically
for the specific type of organism responsible for infection. A careful search for other
underlying diseases is important, looking in particular for ciliary abnormalities, cystic
fibrosis, or immunoglobulin deficiencies.
Treatment
Treatment of bronchiectasis is aimed at controlling infections and bronchial secretions, relieving
airway obstruction, and preventing complications. This includes the prolonged usage of
antibiotics to prevent detrimental infections[18], as well as eliminating accumulated fluid with
postural drainage and chest physiotherapy. Surgery may also be used to treat localized
bronchiectasis, removing obstructions that could cause progression of the disease.[19]
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. One
commonly used therapy is beclometasone dipropionate, which also used in asthma treatment.[20]
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.[21]
Mannitol dry inhalation powder, under the name Bronchitol, has been approved by the FDA for
use in cystic fibrosis patients with or at risk for bronchiectasis. The original orphan drug
indication approved in February 2005 allowed its use for the treatment of bronchiectasis. The
original approval was based on the results of Phase II clinical studies showing the product to be
safe, well-tolerated, and effective for stimulating mucus hydration/clearance, thereby improving
quality of life in patients with chronic obstructive lung diseases like bronchiectasis. Long-term
studies are underway as of 2007 to ensure the safety and effectiveness of the treatment.[22]
Advair Diskus is also a commonly used inhaled corticosteroid which has in many cases been
effective in clearing the airways, reducing sputum and reducing inflammation.


Treatment should involve efforts to resolve any underlying disorder. Infections will
require antibiotics, obstruction may require the removal of a foreign object or tumor.
Medications are available to help thin the sputum, so that it can be more effectively
coughed up. Rhythmic clapping on the chest and back, while the patient assumes a
number of positions (head down, primarily), may help the lungs to drain more effectively.
This is called chest physical therapy, or percussion and postural drainage. In some
patients, bronchiectasis eventually leads to a constantly low level of blood oxygen, despite
other treatments. These patients usually have an associated increase in the size of the
right side of their hearts, along with a decrease in the heart’s ability to pump blood
through the lungs. Some patients with extremely severe symptoms and disability have
been treated with lung transplantation.
Surgery When a particular area of the lung is constantly and severely infected, surgery
may be needed to remove it. When bleeding occurs from irritated bronchial tubes and
overgrown bronchial blood vessels, surgery may be required either to remove an area of
the bronchial tube, or to inject the bleeding blood vessel with a material to stop the
bleeding.
Medicine formularies
Plan or option
GMHPP
Gold Options
G1000, G500 and
G200
Blue Options
B300 and B200
GMISHPP
Link to appropriate Mediscor formulary
[Core]
Blue Option B100
n/a
Epidemiology
Prognosis
Prognosis varies widely, depending on how widespread or focal the bronchiectasis, and the
presence of other underlying disorders.
References
1. Hassan, Isaac (December 8, 2006). "Bronchiectasis". eMedicine Specialties
Encyclopedia. Gibraltar: WebMD. Retrieved on 2007-06-22.
2. Roguin, A (2006). "Rene Theophile Hyacinthe Laënnec (1781–1826): The Man Behind the
Stethoscope" (in English). Clin Med Res 4 (3): 230-35.
3. Wrong O (2003). "Osler and my father" (in English). J R Soc Med 96 (6): 462-64.
doi:10.1258/jrsm.96.9.462.
4. Morrissey BM (2007). "Pathogenesis of bronchiectasis" (in English). Clin Chest Med 28
(2): 289-96. doi:10.1016/j.ccm.2007.02.014. PMID 17467548.
5. Mysliwiec, V, Pina, JS (1999). "Bronchiectasis: the 'other' obstructive lung disease" (in
English). POSTGRADUATE MEDICINE 106 (1): 252-63.
6. Emmons, Ethan (January 31, 2007). "Bronchiectasis". eMedicine Specialties
Encyclopedia. San Antonio, TX: WebMD. Retrieved on 2007-06-22.
7. Morillas HN, Zariwala M, Knowles MR (2007). "Genetic Causes of Bronchiectasis:
Primary Ciliary Dyskinesia" (in English). Respiration 72 (3): 252-63. PMID 17534128.
8. Dalrymple-Hay MJ, Lucas J, Connett G, Lea RE (1999). "Lung resection for the treatment
of severe localized bronchiectasis in cystic fibrosis patients." (in English). Acta Chir
Hung. 38 (1): 23-5. PMID 10439089.
9. Handelsman DJ, Conway AJ, Boylan LM, & Turtle JR (1984). "Young's syndrome.
Obstructive azoospermia and chronic sinopulmonary infections." (in English). NEJM 310
(1): 3-9.
10. Shin MS, Ho KJ (1993). "Bronchiectasis in patients with alpha 1-antitrypsin deficiency. A
rare occurrence?." (in English). Chest 104: 1384-86. doi:10.1378/chest.104.5.1384.
11. Notarangelo LD, Plebani A, Mazzolari E, Soresina A, Bondioni MP (2007). "Genetic
causes of bronchiectasis: primary immune deficiencies and the lung" (in English).
Respiration 74 (3): 264-75. doi:10.1159/000101784. PMID 17534129.
12. Catanzano, Tara (September 5, 2005). "Primary Tuberculosis". eMedicine Specialties
Encyclopedia. Connecticut: WebMD. Retrieved on 2007-06-22.
13. Sheikh S, Madiraju K, Steiner P, Rao M (1997). "Bronchiectasis in pediatric AIDS." (in
English). Chest 112 (5): 1202-7. doi:10.1378/chest.112.5.1202. PMID 9367458.
14. Ferguson HR, Convery RP (2002). "An unusual complication of ulcerative colitis" (in
English). Postgrad. Med. J. 78: 503. doi:10.1136/pmj.78.922.503.
15. Kaushik, VV, Hutchinson D, Desmond J, Lynch MP, and Dawson JK (2004). "Association
between bronchiectasis and smoking in patients with rheumatoid arthritis." (in English).
Annals of the Rheumatic Diseases 63: 1001-2. doi:10.1136/ard.2003.015123.
16. Lamari NM, Martins ALQ, Oliveira JV, Marino LC, Valério N (2006). "Bronchiectasis
and clearence physiotherapy: emphasis in postural drainage and percussion." (in
Portuguese). Braz. j. cardiovasc. surg. 21 (2).
17. Miller, JC (2006). "Pulmonary Mycobacterium Avium-Intracellular Infections in Women"
(in English). Radiology Rounds 4 (2).
18. Evans DJ, Bara AI,Greenstone M (2007). "Prolonged antibiotics for purulent
bronchiectasis in children and adults" (in English). The Cochrane Database of Systematic
Reviews (2). doi:10.1002/14651858.CD001392.pub2.
19. Ötgün B, Karnak B, Tanyel K, Enocak M, Büyükpamukçu N (2003). "Surgical treatment
of bronchiectasis in children." (in English). Journal of Pediatric Surgery 39 (10): 153236.
20. Elborn JS, Johnston B, Allen F, Clarke J, McGarry J, Varghese G. (1992). "Inhaled
steroids in patients with bronchiectasis" (in English). Respir Med 86 (2): 121-4.
doi:10.1016/S0954-6111(06)80227-1. PMID 1615177.
21. Reports, Consumer (2007-03-15). Ipratropium and Albuterol Inhalation - Drug Review.
Consumer Reports of U.S.. Retrieved on 2007-06-22.
22. Waknine, Yael (2005-07-27). Orphan Drug Approvals: Bronchitol, Prestara, GTI-2040.
Medscape today for WebMD. Retrieved on 2007-06-22.
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Prevention." (in English). Br Med J 1 (5490): 783-785.
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(2007). "Analysis of the factors related to mortality in patients with bronchiectasis." (in
English). Respir Med. 101 (7): 1390-97. doi:10.1016/j.rmed.2007.02.002. PMID
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