Brochioectasis

Bronchiectasis
The right clinical information, right where it's needed
Last updated: Mar 21, 2018

Table of Contents
Summary 3
Basics 4
Definition 4
Epidemiology 4
Aetiology 4
Pathophysiology 6
Classification 6
Prevention 8
Primary prevention 8
Secondary prevention 8
Diagnosis 9
Case history 9
Step-by-step diagnostic approach 9
Risk factors 12
History & examination factors 14
Diagnostic tests 15
Differential diagnosis 19
Treatment 21
Step-by-step treatment approach 21
Treatment details overview 24
Treatment options 25
Emerging 31
Follow up 32
Recommendations 32
Complications 32
Prognosis 33
Guidelines 34
Diagnostic guidelines 34
Treatment guidelines 35
References 36
Images 42
Disclaimer 50
Summary
◊ Patients often present with recurrent pulmonary infections, including a chronic daily productive cough
with mucopurulent sputum production.
◊ Diagnostic testing for a potential aetiology of unexplained bronchiectasis should be performed.
◊ A sputum sample should be obtained when the patient is in a stable state and during acute
exacerbations. Systemic antibiotics directed towards prior culture results should be administered.
◊ Daily airway clearance is essential for treatment success.
◊ Maintenance aerosolised antibiotics should be used for treatment of severe bronchiectasis or

recurrent Pseudomonas aeruginosa infections.
◊ Surgical therapy, including lung transplantation, should be considered for patients who continue to
deteriorate despite optimal medical management.
Bronchiectasis Basics
Definition
Bronchiectasis is the permanent dilation of bronchi due to the destruction of the elastic and muscular
components of the bronchial wall.[1] [2] It is often caused as a consequence of recurrent and/or severe
BASICS
infections secondary to an underlying disorder. The majority of patients will present with a chronic cough and
sputum production.[3] [4]
Epidemiology
The prevalence worldwide is unknown due to the lack of standardised medical care and poor health care
access in underdeveloped countries. In the US, an estimated 110,000 individuals are affected, [6] but data
suggest that prevalence is increasing.[7] Bronchiectasis is more common with advancing age, ranging from
4.2 per 100,000 people aged 18 to 34 years to 272 per 100,000 people over 75 years old.[6]
In the UK, the incidence is believed to be 1.06 to 1.3 per 100,000 population.[8] A study in New Zealand
estimated the incidence to be high in children, with a rate of 3.7 per 100,000; incidence varied with ethnicity,
with the highest rate being 17.8 per 100,000 in Pacific children.[9]
There are identifiable causes of bronchiectasis in many patients worldwide, especially bronchiectasis that is
due to prior infection. However, the cause of bronchiectasis may not be identified, and hence the condition is
often considered to be idiopathic. Congenital disorders and immune dysregulation (autoimmune or immune
deficiency) may also cause bronchiectasis.[10]
The following factors have been shown by multivariate analysis to independently predict mortality in patients
with moderate to severe bronchiectasis:[11]
• Pseudomonas aeruginosa infection

• Male sex
• Advanced age
• Higher RV/TLC ratio
• Increased wall thickness on CT imaging
• Low activity level score as judged by St. George’s Respiratory Questionnaire.
Aetiology
Bronchiectasis has many different causes. Recurrent pulmonary infection leads to progressive bronchial
damage. The assessment and determination of an underlying aetiology determine the treatment as well as
prognosis of this disease. The aetiology falls into the following categories:
• Post-infectious:
• Prior childhood respiratory infections due to viruses (i.e., measles, influenza, pertussis)
• Prior infections with Mycobacteria or severe bacterial pneumonia
• Exaggerated response to inhaled Aspergillus fumigatus
[Fig-1]
• Swyer-James or Macleod's syndrome (a chronic manifestation of bronchiolitis or pneumonitis in
childhood, characterised by unilateral pulmonary hypoplasia and radiographic hyperlucency).
• Immunodeficiency:
4 This PDF of the BMJ Best Practice topic is based on the web version that was last updated: Mar 21, 2018.
BMJ Best Practice topics are regularly updated and the most recent version
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• Immunoglobulin deficiency
• HIV infection.
• Genetic:
BASICS
• Cystic fibrosis
• Ciliary dyskinesia or immotile cilia syndrome with or without Kartagener’s syndrome (an
autosomal-recessive condition characterised by the triad of ciliary dyskinesia, situs inversus,
and chronic sinusitis)
• Alpha-1-antitrypsin deficiency.
[Fig-2]
• Aspiration or inhalation injury.
• Connective tissue disorders:
• Rheumatoid arthritis
• Sjogren's syndrome.
• Inflammatory bowel diseases:
• Ulcerative colitis
• Crohn's disease.
• Focal bronchial obstruction:
• Foreign body
• Broncholith
• Stenosis
• Tumour
• Adenopathy with extrinsic compression.
• Other:
• Mounier-Kuhn’s syndrome (also known as tracheobronchomegaly, characterised by abnormal

dilation of the trachea and main bronchi)
• Williams-Campbell’s syndrome (also known as tracheomalacia, characterised by absence or
weakness of bronchial cartilage, leading to bronchial collapse)
• Yellow nail syndrome
• Pulmonary sequestration
• Ehlers-Danlos syndrome
• Marfan’s syndrome
• Young’s syndrome (a condition, believed to be genetic, characterised by obstructive
azoospermia with normal sperm production plus chronic or recurrent sinus and lung infections).
• Idiopathic:
• 7% to 50% of cases.[10] [12]
This PDF of the BMJ Best Practice topic is based on the web version that was last updated: Mar 21, 2018.
5
Pathophysiology
The dilation and thickening of the bronchi seen in bronchiectasis are due to chronic inflammation elicited
by the host response to micro-organisms colonising the airways. This persistent airway inflammation
BASICS
leads to the subsequent development of bronchial wall oedema and increased mucus production. Several
inflammatory cells including neutrophils, T lymphocytes, and other immune effector cells are recruited to
the airways and subsequently release inflammatory cytokines, proteases, and reactive oxygen mediators
implicated in the progressive destruction of the airways.[13] [14] [15]
This initial insult to the airways by the primary infection leads to increased inflammation, resulting in bronchial
damage, and serves as a nidus for subsequent colonisation of the airways. As a result, a vicious cycle
ensues that predisposes to persistent bacterial colonisation and to a subsequent chronic inflammatory
reaction that eventually leads to progressive airway damage and recurrent infections.
The factors that predispose individuals with an initial infection to go on to develop bronchiectasis remain
unclear.
Classification
Morphological classification: Reid classification of
bronchiectasis[2]
Three morphological types were described in 1950 by Dr. Lynn Reid. Though not clinically useful,
classification into morphological types provides insight into pathophysiology and useful descriptive
terminology. Studies have shown that the cystic morphology is more likely to be associated with
Pseudomonas colonisation and more sputum production than the other types.
• Cylindrical bronchiectasis:
• Bronchi enlarged and cylindrical in shape

• Normal tapering of airway as it traverses to the periphery is not present
• Distal airways end abruptly, owing to mucus plugging
• Fewer generations of bronchi than normal
• Parallel tram-track lines are seen on CXR or CT scan
• CT cross-section views reveal 'signet ring' appearance of the dilated bronchus and its
accompanying vessel.
• Varicose bronchiectasis:
• Irregular bronchi, with alternating dilation and constriction

• Bronchographic pattern resembles varicose veins.
• Saccular or cystic bronchiectasis:
• Most severe form

• Commonly found in cystic fibrosis patients
• Bronchi are dilated, forming a cluster of round air-filled or fluid-filled cysts
• Only 25% of the normal number of bronchial subdivisions
• Degree of bronchial dilation increases proximal to distal
• Bronchial tree ends in blind sacs.
BASICS
7
Bronchiectasis Prevention
Primary prevention
Early diagnosis and treatment of predisposing underlying disorders may impede the progression to
bronchiectasis.[12] Smoking cessation and avoidance of second-hand smoke are also important. Influenza
and pneumococcal vaccines are recommended in high-risk populations, in addition to confirmation of
childhood measles (rubeola) and pertussis vaccinations.
Secondary prevention
It is recommended that all patients receive routine vaccinations against Haemophilus influenzae and
Streptococcus pneumoniae .[51] Continuous maintenance treatments to clear mucus and early identification
and treatment of infections and underlying disease aid in reducing the number of exacerbations and
progression in severity.
PREVENTION
Bronchiectasis Diagnosis
Case history
Case history #1
A 55-year-old woman presents for evaluation of a chronic cough, productive of thick, yellow sputum
that sometimes becomes blood-tinged. She has experienced recurrent episodes of fever associated
with pleuritic chest pain. She states that she is embarrassed by the persistent, intractable nature of her
cough and has been prescribed multiple courses of antibiotics. Over the last 5 years, she has developed
shortness of breath with exertion. Her past medical history is significant for pneumonia as a child and
sinus polyps during adulthood for which she has had surgery.
Other presentations
Patients may develop wheezing in addition to increased cough with sputum production, mimicking
chronic bronchitis or asthma. Occasionally, patients may present with haemoptysis. This may be caused
by erosion of the airway wall, chronic inflammation in the context of enlarged bronchial arteries, and
bronchopulmonary anastomoses that are known to occur in bronchiectasis.[5]
Step-by-step diagnostic approach

A high level of suspicion is warranted if characteristic respiratory signs and symptoms accompany an
underlying disorder (e.g., inflammatory bowel disease, connective tissue disorder). Sputum analysis aids
diagnosis; however, high-resolution chest CT is the confirmatory test.
History
Patients typically present with:
DIAGNOSIS
• Persistent productive cough
• Daily mucopurulent sputum
• Dyspnoea[30]
• Fatigue
• Rhinosinusitis
• Blood-tinged sputum (less common)
• Haemoptysis (less common).
An acute exacerbation often presents as worsening of cough, change in sputum colour, increase in
sputum volume, fever, and/or fatigue. More than half of patients with bronchiectasis will have recurrent
episodes of fever, and a history of weight loss is also common.
Physical examination
There may be fever and weight loss. Clubbing of the digits is less common.[30]
Auscultation reveals:
• Crackles
• High-pitched inspiratory squeaks
9
• Rhonchi
• Wheezing.
Pulse oximetry may reveal hypoxaemia in advanced cases of bronchiectasis.
Laboratory investigations
Laboratory tests are geared towards identifying the underlying aetiology, as treatment will be successful
only if it treats both the bronchiectasis and any underlying disease process.
FBC
• The WBC count aids in determining the presence of a superimposed infection or exacerbation.
• If the eosinophil count is high, underlying allergic bronchopulmonary aspergillosis is possible.
Sputum cultures
• Specimens for bacterial, fungal, and acid-fast bacilli cultures are recommended for all patients.
• Single or multiple pathogens may be present.
• Aids in guiding antibiotic selection.
• Referral to a specialist familiar with treatment of non-tuberculous mycobacterium (NTM) is
recommended if culture is positive for an NTM organism.
Sweat sodium chloride concentration and genetic testing for cystic fibrosis transmembrane conductance
regulator (CFTR) mutation analysis
• Recommended for all children and for adults in whom there is a high index of suspicion since
patients may present with variant forms of cystic fibrosis in adulthood.
• Minimum testing should include 2 measurements of sweat chloride and then CFTR mutation
analysis.
• If concentration of sodium chloride in patient's sweat is high, a diagnosis of cystic fibrosis is very
likely.
• Factors for a high index of suspicion for cystic fibrosis in adults would include:[31]
DIAGNOSIS
• Age at presentation is over 40 years and no other identified cause

• Persistent isolation of S. aureus
• Features of malabsorption
• Male primary infertility
• Upper lobe bronchiectasis
• A history of childhood steatorrhoea.
Serum alpha-1 antitrypsin phenotype and level
• Recommended to identify alpha-1 antitrypsin disease as underlying aetiology.

• If abnormal result, referral to a pulmonary specialist for consideration of replacement therapy is
indicated.
Immunoglobulin levels (serum total IgG, IgM, IgA, and IgE), IgG subclasses (IgG1, IgG2, IgG3, IgG4),
and response to Pneumovax vaccine with Strep pneumo 23 serotype titres.[32]
• Recommended to identify individual immunoglobulin deficiencies as underlying aetiology.

Skin prick test for sensitivity to Aspergillus fumigatus for patients with elevated IgE
• Recommended in patients with asthma, as a first step in evaluation of patients with suspected
allergic bronchopulmonary aspergillosis.
• If positive, serum precipitins to Aspergillus should be measured.
• A diagnosis of allergic bronchopulmonary aspergillosis warrants referral to a pulmonary specialist.
HIV antibody test
• Recommended in all patients.

• Patients with HIV infection are predisposed to developing recurrent sinopulmonary infections and
bronchiectasis, which is likely to be due to abnormal B-lymphocyte function.[33]
Rheumatoid factor

• Bronchiectasis is more common in rheumatoid arthritis population versus general population.
• Bronchiectasis symptoms may precede other systemic rheumatoid arthritis findings.[26]
Nasal nitric oxide test
• Recommended in patients with a history of frequent childhood sinopulmonary infections to evaluate

for primary ciliary dyskinesia (PCD).
• A low result (<100 parts per billion) warrants nasal brushing to obtain ciliated epithelium to evaluate
ciliary motion (video microscopy) and ultrastruction (electron microscopy). Genetic testing for PCD
is done at research centres.
Radiographical studies
CXR is not recommended for diagnosis as CXR findings are non-specific in bronchiectasis and may even
be normal. Nevertheless, large lung volumes, areas of consolidation (due to impacted mucus), tram lines,
and tubular or ovoid opacities may be present. Thin-walled ring shadows with or without fluid levels may
be present as well.
[Fig-3]
DIAGNOSIS
[Fig-4]
High-resolution CT scan is the best diagnostic tool for bronchiectasis. Axial CT sections show dilation
of bronchi with or without airway thickening. In cross-section, the dilated bronchus will be wider than its
accompanying pulmonary artery and resemble a signet ring. Abnormal bronchial dilation is recognised
as lack of normal tapering, producing a tram line or flared appearance in the periphery of the lung, and is
more prominent when the bronchial walls are thickened. Affected small airways plugged by mucus appear
as small, irregular, V- and Y-shaped markings and are about 2 mm in size in the periphery of the lung.
This is referred to as a tree-in-bud pattern.[34]
[Fig-5]
[Fig-6]
[Fig-7]
[Fig-8]
11
Pulmonary function tests
Spirometry is recommended with most surgery visits. Obstructive airways disease may be evidenced
by reduced forced expiratory volume (FEV1) or an FEV1/forced vital capacity (FVC) ratio of <70%.[30]
Reduction of the FEV1 suggests the presence of infection or worsening bronchiectasis.
Full pulmonary function testing may show an elevation of RV/TLC ratio consistent with air trapping.
Diffusing capacity for carbon monoxide (DLCO) may be reduced in severe disease.
Additional work-up
A swallow study or pH monitoring to evaluate for chronic aspiration is also suggested. If these suggest the
presence of aspiration, referral to a gastroenterologist and/or ENT specialist is recommended.
A 6-minute walk test should be considered for patients at risk of desaturation with exercise.
Risk factors
Strong
cystic fibrosis
• Most common identifiable cause of bronchiectasis.
• A diagnosis of cystic fibrosis should be considered in younger adults with recurrent sinopulmonary
infection and/or bronchiectasis, even in the absence of GI symptoms.[3]
• Although primarily diagnosed in early childhood, an initial presentation of recurrent sinopulmonary
infections in adulthood has become more frequent. Cystic fibrosis patients diagnosed later in life
tend to have less pancreatic insufficiency, leading to a reduced likelihood of diabetes and better lung
function than patients diagnosed early in life.[16]
• Typically presents with sinusitis and bronchiectasis.
• Typical colonising organisms include Pseudomonas aeruginosa or Staphylococcus aureus .
• Mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene lead to
DIAGNOSIS
abnormalities of the chloride channels causing viscous mucus and abnormal airway clearance.
host immunodeficiency
• Includes immunoglobulin deficiency and HIV infection.
• Deficiencies in humoral immunity (either congenital or acquired) associated with decreased levels of
IgG (or IgG subclasses), IgM, or IgA are shown to lead to increased risk of sinopulmonary infections
and bronchiectasis.[17]
• Leads to diffuse disease.
• Treatment with IV immunoglobulin replacement has been shown to decrease infection rate and the
progression of bronchiectasis.[18]
previous infections
• Associated with bronchial destruction resulting in bronchiectasis.
• Patients with long-standing asthma may develop an exaggerated hyperimmune response to inhaled
Aspergillus fumigatus , resulting in chronic airway damage.[19]
• Mycobacterium avium-intracellulare complex may colonise the airways of normal hosts, primarily
women over 50 years.[20]
• Bronchiectasis may occur following virus infections (i.e., measles, influenza, pertussis) or due to prior
infections with Mycobacteria tuberculosis or severe bacterial pneumonia.
• Swyer-James or Macleod's syndrome are forms of lung hypoplasia occurring secondary to childhood
infection such as adenovirus.
congenital disorders of the bronchial airways

• Patients with Young's syndrome exhibit similar features to those of cystic fibrosis patients (including
bronchiectasis, sinusitis, and obstructive azoospermia) but without a CFTR genetic mutation or
positive sweat chloride test.[21]
• Other conditions associated with bronchiectasis include Mounier-Kuhn syndrome
(tracheobronchomegaly), Williams-Campbell syndrome (cartilage deficiency), pulmonary
sequestration, and yellow nail syndrome.[3]
primary ciliary dyskinesia

• An autosomal-recessive disorder with a defect in ciliary structure and/or function. The dysfunctional
cilia lead to recurrent ear, sinus, and lung infections.
• Kartagener's syndrome includes the triad of bronchiectasis, sinusitis, and situs inversus, and occurs in
approximately 50% of patients with primary ciliary dyskinesia.[22] [23] [24]
Weak
alpha-1 antitrypsin deficiency
• A genetic disorder that predisposes to the development of COPD and, less commonly, bronchiectasis.
The estimated prevalence of bronchiectasis in these patients is between 2% and 43%.[25]
connective tissue disease

• Bronchiectasis occurs in 1% to 3% of rheumatoid arthritis (RA) patients. Although typically a late
finding, one study showed that bronchiectasis preceded the systemic complications of RA in 20% of
cases.[26]
• Other diseases that may lead to bronchiectasis are Ehlers-Danlos syndrome, Marfan's syndrome, and
DIAGNOSIS
Sjogren's syndrome.
inflammatory bowel disease

• Bronchiectasis occurs more commonly in patients with ulcerative colitis than in those with Crohn's
disease.[27]
• May be due to increased infiltration of immune effector cells into the airways.[28]
aspiration or inhalation injury

• Blockage and tissue damage increase risk.
focal bronchial obstruction

• Includes broncholith, stenosis, tumour, and adenopathy with extrinsic compression.
tall, thin, white females, aged 60 or over

• Are at greater risk of pulmonary non-tuberculous mycobacteria (PNTM)-related bronchiectasis. Mitral
valve prolapse, scoliosis, and heterozygous cystic fibrosis transmembrane conductance regulator
(CFTR) mutations have been found in greater frequency in these patients.[29]
13
History & examination factors

Key diagnostic factors
presence of risk factors (common)
• Risk factors include cystic fibrosis (the most common identifiable cause of bronchiectasis),
immunodeficiency, previous infections, congenital disorders of the bronchial airways, primary ciliary
dyskinesia, alpha-1 antitrypsin deficiency, connective tissue disease, inflammatory bowel disease,
aspiration or inhalational injury, and focal bronchial obstruction.
cough (common)
• Occurs in 98% of patients and is the most common symptom of bronchiectasis. An acute exacerbation
often presents as worsening of cough.
• May be associated with large amounts of purulent sputum and, less commonly, haemoptysis.
• Cough may be worsened by lying flat or on one side.
sputum production (common)

• Daily sputum production is present in two-thirds of patients. Bloody sputum is present in about 50% of
patients and is usually mild (i.e., sputum with flecks of blood).
• Sputum production will often increase during acute respiratory infections.
• An acute exacerbation often presents as a change in sputum colour and an increase in sputum
volume.
crackles, high-pitched inspiratory squeaks and rhonchi (common)

• Many patients will have crackles on pulmonary auscultation. High-pitched inspiratory squeaks and
pops are commonly heard.
dyspnoea (common)
• Present in majority of patients, especially with exertion.
DIAGNOSIS
• Often correlates with severity of bronchiectasis on chest CT.
fever (common)
• More than half of patients with bronchiectasis will have recurrent episodes of fever.
• An acute exacerbation often presents with fever.
Other diagnostic factors

fatigue (common)
• A non-specific symptom but very common symptom. An acute exacerbation often presents with
fatigue.
haemoptysis (common)
• Present in about 50% of patients and is usually mild (i.e., sputum with flecks of blood).
• Thought to originate from the bronchial arteries or bronchial-pulmonary anastomoses.
• May become massive (>250 mL/day), which warrants hospital admission and immediate referral to a
pulmonologist and/or a thoracic surgeon or interventional radiologist for bronchial artery embolisation.
rhinosinusitis (common)
• Bronchiectasis due to a primary mucociliary clearance defect, such as primary ciliary dyskinesia or
cystic fibrosis, will probably be accompanied by symptoms of rhinosinusitis.
weight loss (common)

• A non-specific sign.
wheezing (common)
• Present in one quarter of patients, but more common in bronchiectasis patients who have concomitant
asthma.
pleuritic chest pain (uncommon)

• May be present, especially during periods of exacerbation.
clubbing (uncommon)
• An abnormal angle (>180°) between the nail bed and the finger.
• Clubbing of the digits is rare.[30]
Diagnostic tests
1st test to order
Test Result
CXR may be normal or show
obscured hemidiaphragm,
• Findings are non-specific and often non-diagnostic, but may show
thin-walled ring shadows
characteristic volume loss obscuring the underlying hemidiaphragm,
with or without fluid
tram lines, and tubular or ovoid opacities.
• Thin-walled ring shadows with or without fluid levels may also be levels, tram lines, tubular
or ovoid opacities
present.
• Although chest CT scan is the diagnostic procedure of choice, CXR
DIAGNOSIS
(PA and lateral) is sufficient for subsequent monitoring.
[Fig-3]
[Fig-4]
15
Test Result
high-resolution chest CT thickened, dilated airways
with or without air
• Recommended for all patients.
fluid levels; varicose
• Shows dilation of bronchi with or without airway thickening.
constrictions along
[Fig-8]
airways; cysts and/or tree-
• In cross-sectional appearance, the bronchi are larger than their in-bud pat tern
adjacent pulmonary artery (signet ring sign).[35]
[Fig-6]
[Fig-7]
• Abnormal bronchial dilation is recognised as the lack of normal
tapering of bronchi, producing a tram line or flared appearance in the
periphery of the lung; more prominent when the bronchial walls are
thickened.
• Affected small airways plugged by mucus appear as small, irregular,
V- and Y-shaped markings 2 mm in size in the periphery of the lung
(tree-in-bud pattern).
[Fig-5]
• May show fluid-filled cysts; these represent superimposed infection
and warrant a course of systemic antibiotics.
FBC WBC differential
may reveal high
• The WBC count will aid in determining the presence of a
eosinophil count in
superimposed infection.
bronchopulmonary
• If the eosinophil count is high, an underlying allergic
bronchopulmonary aspergillosis is possible. aspergillosis;
neutrophilia suggests
superimposed infection or
exacerbation
DIAGNOSIS
Test Result
sputum culture and sensitivity gram-positive bacteria;
gram-negative bacteria;
• Frequently, a pathogenic organism can be recovered in the sputum.
non-tuberculous
There may be single or multiple pathogens present. The most
mycobacteria; fungi
common gram-negative organism is Pseudomonas aeruginosa ,
present in about 25% of patients; it may be in mucoid form. Mucoid
P aeruginosa has been shown to correlate with lower pulmonary
function and is a marker for severe lung damage.[36] [37] It requires
special attention because it is more likely to be resistant to antibiotics
than non-mucoid Pseudomonas species.[38]
• Specimens for bacterial culture may be requested as 'cystic fibrosis
bacterial culture' in patients who are at high risk of Pseudomonas or
who have had Pseudomonas growth in sputum cultures in the past.
This will allow for special vigilance for mucoid-type Pseudomonas
, which has been shown to correlate with lower pulmonary function.
A cystic fibrosis bacterial culture employs separate agars to select
for gram-negative bacterial growth, as well as Burkholderia cepacia
. Sensitivities for these cultures are done by manual disc diffusion
because mucoid Pseudomonas does not grow well by automated
methods.
• Gram-positive cocci ( Staphylococcus aureus , Streptococcus
pneumoniae , or beta-haemolytic streptococci) represent about 18%
of positive sputum cultures.
• Other common gram-negative organisms are Haemophilus
influenzae , Klebsiella pneumonia , Moraxella catarrhalis , and
Serratia marcescens . Mycobacteria are commonly isolated as well.
• Infectious disease consultation may be needed if non-tuberculous
mycobacteria is present in sputum cultures.[30]
serum alpha-1 antitrypsin phenotype and level may be abnormal
phenotype or level
• Referral for consideration of replacement therapy is suggested if
abnormal phenotype or level.
• The MM phenotype indicates patient is homozygous for the normal M
allele.[39]
DIAGNOSIS
serum immunoglobulins decreased IgG, IgM, and/
or IgA with inappropriate
• Functional assessment of immunoglobulins is performed by testing
titres to Pneumovax
titres to Strep pneumo 23 serotypes in response to Pneumovax.[32]
Immunoglobulin replacement reduces the frequency of infectious
episodes and prevents further destruction of the airways.
sweat chloride test >60 mmol/L (>60 mEq/L)
cystic fibrosis is likely;
• An abnormal test is diagnostic for cystic fibrosis.
40 to 59 mmol/L cystic
• If abnormal or intermediate, cystic fibrosis transmembrane regulator
fibrosis is possible; <39
protein (CFTR) gene mutation analysis should be done.
mmol/L cystic fibrosis is
very unlikely
rheumatoid factor positive rheumatoid factor

may be detected
• The prevalence of bronchiectasis is increased in patients with
rheumatoid arthritis compared with the general population.
• The symptoms of bronchiectasis may precede other systemic findings
in rheumatoid arthritis.[26]
17
Test Result
Aspergillus fumigatus skin prick test immediate cutaneous
reactivity to antigen
• Recommended in patients with central bronchiectasis, asthma, and
in bronchopulmonary
elevated IgE.
aspergillosis
• Further testing should include total serum IgE and Aspergillus
fumigatus IgG precipitins.
serum HIV antibody positive in HIV infection
• Patients with HIV infection are predisposed to developing recurrent
sinopulmonary infections and bronchiectasis, which is probably due
to abnormal B-lymphocyte function.[33]
nasal nitric oxide (NNO) low (<100 parts per billion)
NNO level in primary
• Recommended for patients with a high clinical suspicion of primary
ciliary dyskinesia; high
ciliary dyskinesia (PCD).
NNO level excludes a
• Sensitivity of 97% and specificity of 90% for PCD.
• A low NNO should be followed up with confirmatory testing (nasal diagnosis of PCD
or bronchial brush biopsy for ciliary examination) because other
conditions such as cystic fibrosis may present with low NNO.
• A high NNO virtually excludes PCD.
pulmonary function tests reduced FEV1, elevated
RV/TLC
• Reduction of the FEV1 suggests the presence of infection or
worsening bronchiectasis.
• Spirometry recommended with most surgery visits.
Other tests to consider
Test Result
bronchial biopsy and electron microscopy of cilia abnormal ciliary
beat motion and/or
• Can be used to confirm a low nasal nitric oxide and, therefore,
morphology in primary
diagnose primary ciliary dyskinesia.
DIAGNOSIS
• Nasal or bronchial tissue is analysed by video microscopy to evaluate ciliary dyskinesia

ciliary beat motion and, under electron microscopy, to evaluate ciliary
structure.
• Lipid-laden macrophages seen in bronchial biopsy specimens
suggests aspiration.
cystic fibrosis transmembrane regular (CFTR) protein gene positive in cystic fibrosis
mutation testing
• If the sweat chloride test is abnormal, the patient should undergo
CFTR gene mutation testing.
swallow study may be abnormal in
presence of chronic
• Suggested to evaluate for chronic aspiration.
aspiration
• If a swallow study or pH monitoring suggests the presence of
aspiration, referral to a gastroenterologist is recommended.
sputum pH monitoring low pH in patients with
chronic aspiration
• Suggested to evaluate for chronic aspiration. May be done by a
gastroenterologist in some centres.
• If a swallow study or pH monitoring suggests the presence of
aspiration, referral to a gastroenterologist is recommended.
Test Result
6-minute walk test reduced in those with
significantly reduced lung
• Should be considered for patients at risk for desaturation with
function
exercise. Can be used to assess serial changes in functional
capacity.
Differential diagnosis
Condition Differentiating signs / Differentiating tests

symptoms
COPD • Diminished breath sounds, • Chest CT may be normal
characterising COPD, are or show emphysema
not found in bronchiectasis. in COPD, as opposed
• In bronchiectasis, rhonchi to the characteristic
may be auscultated, but abnormal results found in
with additional inspiratory bronchiectasis (thickened,
squeaks and crackles. dilated airways with or
without air fluid levels,
varicose constrictions along
airways, ballooned cysts
at the end of a bronchus,
and/or tree-in-bud pattern).
Patients with COPD may
also develop bronchiectasis.
asthma • Inspiratory squeaks and • Chest CT may show

crackles, often present thickened airways but lack
in bronchiectasis, are not the enlarged or widened
present in asthma. airway (signet ring sign)
seen in bronchiectasis
(thickened, dilated airways
with or without air fluid
DIAGNOSIS
levels, varicose constrictions
along airways, ballooned
cysts at the end of a
bronchus, or tree-in-bud
pattern).
• Airflow obstruction is often
reversible in asthma.
19
Condition Differentiating signs / Differentiating tests

symptoms
pneumonia • Patients with pneumonia • CXR and chest CT results
describe symptoms of short in pneumonia are quite
duration (7 to 10 days), variable and often depend
as opposed to years in on aetiology.
bronchiectasis. • In bronchiectasis, there
• Auscultation findings is characteristic dilation
(rhonchi, wheezing, of bronchi with or without
crackles) may be similar airway thickening.
in bronchiectasis and • Consolidation, which is seen
pneumonia, especially in pneumonia, is not seen in
multi-lobar pneumonia. bronchiectasis.
Bronchial breath sounds,
which are characteristic of
pneumonia, are not present
in bronchiectasis.
chronic sinusitis • The inspiratory squeaks • CXR and chest CT

and crackles found are normal in chronic
in bronchiectasis are sinusitis. Sinus CT shows
uncommon in chronic opacification of the involved
sinusitis. facial sinus in chronic
sinusitis.
DIAGNOSIS
Bronchiectasis Treatment
Step-by-step treatment approach

Treatment for all patients involves maintenance therapy and treatment of acute exacerbations, with attention
to the specific organism involved. For a select group of patients, surgery may be indicated. Patients with
severe disease or a resistant organism (typically Pseudomonas ) are likely to require IV antibiotics during
acute exacerbations. For severe, progressive disease lung transplantation should be considered.
The goal of treatment is to decrease baseline clinical symptoms of cough, sputum production, and dyspnoea,
to improve quality of life, to decrease the number of acute exacerbations, and to prevent progression of
airway damage. The particular organism present in the sputum, specifically Pseudomonas , and the
frequency of exacerbations determine antibiotic treatment choices. Those with low risk of developing a
Pseudomonas infection have few, if any, exacerbations and may require less maintenance therapy.
The number of studies assessing treatments for non-cystic fibrosis bronchiectasis is increasing, therefore
evidence for specific treatments in this area is building. More randomised controlled trials are needed
with an endpoint focused on reducing exacerbations and improving quality of life, rather than reducing
sputum volume. Additionally, treatment of any identified underlying disease (e.g., cystic fibrosis) or comorbid
disease (e.g., gastro-oesophageal reflux disease) is paramount. Mucolytics, specifically rhDNase, are not
recommended in patients with non-cystic fibrosis bronchiectasis.
Maintenance therapy for all patients

Airway clearance is impaired in patients with bronchiectasis, and bronchial hygiene to loosen secretions
and enhance their removal is the mainstay of maintenance therapy.[31] A systematic review of airway
clearance techniques in people with non-cystic fibrosis bronchiectasis concluded that airway clearance
techniques seem to be safe in people with stable bronchiectasis. They may be associated with
improvements in sputum expectoration, selected measures of lung function, symptoms, and health-
related quality of life scores. However, it is not clear what role they play in the management of acute
bronchiectasis.[40]
Airway clearance includes:
• Maintenance of oral hydration.

• Chest physiotherapy (performed concurrently with the nebulised agents), including postural
drainage, percussion, and vibration with the use of oscillatory devices such as a flutter valve and
the vibration vest.[41]
• Use of nebulised hyperosmolar agents such as hypertonic saline or mannitol (agents intended to
promote mucus clearance by inducing coughing). Nebulised hypertonic saline has been shown to
reduce inflammatory mediators, improve sputum bacteriology, and improve quality of life scores.[42]
[43] Hypertonic saline may cause chest tightness and wheezing in some patients. Mannitol is of
uncertain clinical benefit;[44] it failed to reduce exacerbation rates in a large, randomised controlled
trial.[45] [46] Bronchodilators should be used prior to administration of nebulised hyperosmolar
agents.
• Other mucolytic agents that may be beneficial in a subset of patients are N-acetylcysteine,
TREATMENT
erdosteine, and bromhexine.[47] [48]

Airway clearance is generally recommended for 15 to 30 minutes, 2 or 3 times daily. Many of these
interventions require the assistance of a carer. The therapy is time-consuming, and patients often find the
process unpleasant.[49] Patient preference should weigh heavily in which technique is chosen.
21
Diet and exercise are important components of therapy:
• A healthy diet and exercise are recommended in all patients.

• Exercise is considered a form of airway clearance.
• Pulmonary rehabilitation consisting of an 8-week training programme at 80% peak heart rate has
been shown to improve exercise and endurance capacity.[50]
• A higher BMI has been shown to correlate with beneficial outcome.[51]
The results of several studies suggest that oral macrolides are of therapeutic benefit in bronchiectasis
patients with or without Pseudomonas .[52] [53] [54] Benefits include a small improvement in FEV1, a
decrease in sputum volume, and decreased exacerbation rate. However, vigilance is required. Presence
of mycobacteria in the sputum necessitates prompt discontinuation of macrolide monotherapy to minimise
the risk of resistance developing. The dose, specific macrolide, and duration of therapy (months to
indefinitely) are not completely established.
Maintenance therapy for select patients

Bronchodilators can improve the tolerability of hyperosmolar agents used to induce coughing, although
supporting evidence is weak. They are appropriate in patients with co-existing asthma or COPD.[31]
Repeated exacerbations, a recent history of antibiotic use, and cystic fibrosis are risk factors for
Pseudomonas infection. Case records suggest that early, aggressive antibiotic therapy may eradicate
Pseudomonas infection in some patients with bronchiectasis, but this is yet to be confirmed in a
prospective, randomised trial.[55]
For patients with Pseudomonas infection or with frequent exacerbations, macrolide antibiotics given 3
times weekly may lead to reduced symptoms, reduced exacerbations, and improved lung function.[31]
Most patients with bronchiectasis are chronically infected with bacteria, even during the stable state,
and their bacterial load correlates with markers of inflammation. Treatment with inhaled gentamicin for a
12-month period decreased markers of inflammation and the risk of subsequent exacerbations in these
patients.[56] [57]Inhaled antibiotics may:
• Produce small benefits in reducing sputum volume and purulence, but be most useful in the
treatment of underlying chronic infection
• Be associated with intolerable adverse effects (wheezing and coughing) and the emergence of
resistance.
Sensitivity must be confirmed prior to initiation of inhaled antibiotics. Bronchodilators should be used prior
to administration of inhaled antibiotics.
Prolonged use of antibiotics (4 weeks or more) has been associated with a reduced risk of exacerbations
and hospitalisations in patients with bronchiectasis; however, there are concerns regarding the increased
risk of emerging drug resistance.[58] The long-term use of fluoroquinolones in the treatment of respiratory
infections in patients with bronchiectasis may mask active pulmonary tuberculosis. Vigilant mycobacterial
surveillance is important in this patient population.[59]
TREATMENT
Inhaled corticosteroids have been shown to decrease 24-hour sputum volume and to lessen
exacerbations.[60] Large and long-term supportive studies, however, are lacking.
Treatment of exacerbations
An acute exacerbation typically presents as worsening of cough, change in sputum colour, increase in
sputum volume, fever, or malaise. Antibiotics are the mainstay of treatment and should be selected for
their activity against likely pathogens. For patients who have never had an exacerbation and never had
Pseudomonas in their sputum cultures, an appropriate initial choice would be an antibiotic with coverage
against Haemophilus influenzae or Staphylococcus aureus , depending on the culture results.[31]
• For those patients suitable for outpatient care, oral antibiotic treatment (e.g., amoxicillin,
clarithromycin, or trimethoprim/sulphamethoxazole) is appropriate for 14 days.
• For patients with known Pseudomonas , moderate to severe disease, or frequent exacerbations,
intravenous antibiotic treatment with an aminoglycoside or a fluoroquinolone may be needed for at
least 14 days.[3]
Airway clearance to clear mucus, with or without bronchodilators, is important and should be increased in
frequency for patients of any disease severity during the treatment of exacerbations. Concurrent treatment
of the underlying infection is essential. There is no evidence to support use of systemic corticosteroids
in the treatment of acute exacerbations in either mild or severe disease with or without Pseudomonas
species.
Treatment of refractory cases

Complete resection of bronchiectatic areas of the lung may be appropriate in some patients with
refractory disease:
• It is appropriate for patients with severe, focal disease in 1 or both lobes of a lung and with limited
success with antibiotic therapy. Complete resection of the bronchiectatic area is associated with
the best results. Advanced age and renal failure are associated with increased postoperative
complications.[61] [62]
• Surgery may be indicated for massive haemoptysis and, possibly, in treatment of focal non-
tuberculous mycobacteria (NTM) or Aspergillus species.
Long-term ox ygen therapy

The evidence regarding administration of non-invasive ventilation is poor, but there may be some benefit
in patients with severe ventilatory failure with reduced number of hospital admissions.[31] Supplemental
oxygen therapy may be given as required. Oxygen saturation must be carefully monitored to prevent
hypercapnic respiratory failure.
Lung transplant
Patients with the following criteria should be considered for referral to a lung transplant centre:
• FEV1 <30% predicted or rapid decline in FEV

• Exacerbation that requires an ICU stay
• Increasing frequency of exacerbations that require antibiotic therapy
TREATMENT
• Refractory or recurrent pneumothorax

• Recurrent haemoptysis not controlled by embolisation.
Patients with oxygen-dependent respiratory failure, hypercapnia, or pulmonary hypertension should also
be considered for lung transplant.[63]
23
Treatment details overview

Please note that formulations/routes and doses may differ between drug names and brands, drug
formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer
Ongoing ( summary )
all patients
1st exercise and improved nutrition
plus airway clearance therapy
adjunct inhaled bronchodilator
adjunct inhaled hyperosmolar agent
adjunct long-term oral macrolide
with high risk for plus inhaled antibiotic

or known chronic
Pseudomonas infection
with acute exacerbation: plus short-term oral antibiotic

mild to moderate disease
with acute exacerbation: plus short-term intravenous antibiotic

severe or not responding/
resistant to oral
antibiotics
with recurrent infections, adjunct surgery

severe haemoptysis, or
focal disease
with severe ventilatory plus non-invasive ventilation

failure
adjunct supplemental ox ygen

TREATMENT
Treatment options
Please note that formulations/routes and doses may differ between drug names and brands, drug
formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer
Ongoing
all patients
1st exercise and improved nutrition
» A healthy diet and exercise are recommended

for all patients. Pulmonary rehabilitation,
consisting of an 8-week training programme
at 80% of peak heart rate, has been shown to
improve exercise and endurance capacity.[50] In
addition, exercise helps with mucus clearance.
» A higher BMI has been shown to correlate with

a beneficial outcome.[51]
plus airway clearance therapy
Treatment recommended for ALL patients in
selected patient group
» Airway clearance therapy includes postural
drainage, percussion, vibration, and the
use of oscillatory devices. A primary goal is
bronchopulmonary hygiene.
» Therapy is generally recommended for 15 to

30 minutes, 2 or 3 times daily. Many of these
interventions require the assistance of a carer.
The therapy is time-consuming, and patients
often find the process unpleasant.[49] Patient
preference should be strongly considered in
which technique is chosen.
adjunct inhaled bronchodilator
Treatment recommended for SOME patients in
Primary options
» salbutamol inhaled: 200 micrograms (2

puffs) every 4-6 hours when required; 2.5 mg
nebulised every 6-8 hours when required
OR
» arformoterol inhaled: 15 micrograms

nebulised every 12 hours when required
TREATMENT
OR
» ipratropium inhaled: 40 micrograms (2

puffs) every 6 hours when required
OR
25
Ongoing
» tiotropium inhaled: 18 micrograms (1

capsule) inhaled once daily when required
» Nebulised bronchodilators given before

therapy with hyperosmolar agents may improve
tolerability, especially in patients with concurrent
asthma or chronic obstructive pulmonary
disease, although the evidence for their use is
weak.
adjunct inhaled hyperosmolar agent
Primary options
» hypertonic saline inhaled
OR
» mannitol inhaled: consult specialist for

guidance on dose
» Nebulised hypertonic saline has been shown

to reduce inflammatory mediators, improve
sputum bacteriology, and improve quality of life
scores.[42] [43] It may cause chest tightness
and wheezing in some patients. Bronchodilators
should be used prior to administration.
adjunct long-term oral macrolide
Primary options
» azithromycin: 250 mg orally once daily, or

500 mg orally three times weekly
» The results of several studies suggest that

oral macrolides are of therapeutic benefit
in bronchiectasis patients with or without
Pseudomonas .[52] [53] [54] Benefits include a
small improvement in FEV1, decreased sputum
volume, and decreased exacerbation rate.
However, vigilance is required. Presence of
mycobacteria in the sputum necessitates prompt
discontinuation of macrolide monotherapy to
minimise the risk of resistance developing. The
dose, specific macrolide, and duration of therapy
(months to indefinitely) are not completely
TREATMENT
established.
» For patients with Pseudomonas infection

or with frequent exacerbations, macrolide
antibiotics given 3 times weekly may lead to
Ongoing
reduced symptoms, reduced exacerbations, and
improved lung function.[31]
with high risk for plus inhaled antibiotic
or known chronic
Pseudomonas infection
Primary options
» tobramycin inhaled: 300 mg nebulised

every 12 hours; give in cycles of 28 days on
and then 28 days off
OR
» colistimethate sodium: dose depends

on local formulation; consult specialist for
guidance on dose
OR
» gentamicin: 80 mg nebulised every 12

hours (no cycling)
» Repeated exacerbations, recent history of

antibiotic use, and cystic fibrosis are risk factors
for Pseudomonas infection.
» Most patients with bronchiectasis are

chronically infected with bacteria, even during
the stable state. The bacterial load present
in these patients has been shown to directly
correlate with markers of inflammation.
Treatment of these patients with alternating
months of inhaled gentamicin for a 12-month
period was shown to decrease markers
of inflammation and decrease the risk of
subsequent exacerbations.[56]
» Inhaled antibiotic options include

tobramycin,[64] colistimethate,[65] and
gentamicin.[56] [57]
» This treatment has been shown to improve

respiratory symptoms in patients with severe
bronchiectasis, but some patients also suffered
from adverse symptoms such as cough,
wheezing, and fatigue in response to the
treatment.[66] It may also be associated with the
emergence of resistance.
» Sensitivity must be confirmed prior to initiation

TREATMENT
of inhaled antibiotics. Bronchodilators should be

used prior to administration of inhaled antibiotics.
with acute exacerbation: plus short-term oral antibiotic
mild to moderate disease
27
Ongoing
Primary options
» amoxicillin: 500-875 mg orally twice daily
OR
» clarithromycin: 500 mg orally twice daily
OR
» trimethoprim/sulfamethoxazole: 160/800 mg
orally twice daily
OR
» ciprofloxacin: 500-750 mg orally twice daily
OR
» levofloxacin: 750 mg orally once daily
» An acute exacerbation typically presents as

worsening of cough, change in sputum colour,
increase in sputum volume, fever, or malaise.
» Antibiotics are the mainstay of treatment and

should be selected for their activity against likely
pathogens.
» For patients who have never had an

exacerbation, and never had Pseudomonas
in their sputum cultures, an appropriate
initial choice would be an antibiotic with
coverage against Haemophilus influenzae
or Staphylococcus aureus , depending on the
culture results.[31]
» For those patients with low likelihood of

Pseudomonas , oral antibiotic treatment is
appropriate.
» In patients with mild to moderate symptoms

and a known Pseudomonas infection and
symptoms of a bronchiectasis exacerbation,
antibiotics should be directed towards
Pseudomonas species sensitivities.
» Sensitivity to fluoroquinolones must be

confirmed when using oral therapy.
TREATMENT
» Treatment course: 14 days.

with acute exacerbation: plus short-term intravenous antibiotic
severe or not responding/
resistant to oral
antibiotics
Ongoing
Primary options
» ciprofloxacin: 400 mg intravenously every

12 hours
OR
» cefepime: 2 g intravenously every 12 hours
OR
» vancomycin: 1 g intravenously every 12

hours
OR
» gentamicin: 3-6 mg/kg/day intravenously

given in divided doses every 8 hours
» An acute exacerbation typically presents as

worsening of cough, change in sputum colour,
increase in sputum volume, fever, or malaise.
» Antibiotics are the mainstay of treatment and

should be selected for their activity against likely
pathogens.
» Patients with severe disease or a resistant

organism (typically Pseudomonas ) are likely
to require intravenous antibiotics during acute
exacerbations.
» An appropriate initial choice would be

an antibiotic with coverage against prior
culture results. Cefepime may be used
for patients with known Pseudomonas
resistant to fluoroquinolones. Vancomycin is
appropriate for patients with methicillin-resistant
Staphylococcus aureus (MRSA). Gentamicin,
if used, should be used cautiously with close
monitoring of renal function and serum levels.
Vancomycin also requires monitoring of serum
levels.
» Treatment course: 14 days.

with recurrent infections, adjunct surgery
severe haemoptysis, or
focal disease
» Complete resection of bronchiectatic areas of
TREATMENT
the lung may be appropriate in some patients

with refractory disease.
» Surgery may be appropriate for patients with

severe, focal disease in one or both lobes of a
lung and limited success with antibiotic therapy.
29
Ongoing
It is also indicated for massive haemoptysis, and
possibly in treatment of focal non-tuberculosis
mycobacteria (NTM) or Aspergillus species.
» Lung transplantation should be considered in

patients with FEV <30% of predicted or a rapid
decline in FEV, an exacerbation requiring an
ICU stay, increasing frequency of exacerbations
requiring antibiotic therapy, refractory or
recurrent pneumothorax, recurrent haemoptysis
not controlled by embolisation, oxygen-
dependent respiratory failure, hypercapnia, or
pulmonary hypertension.[63]
with severe ventilatory plus non-invasive ventilation
failure
» The evidence is poor, but there may be some
benefit in this patient group with reduced number
of hospital admissions.[31]
adjunct supplemental ox ygen
» Oxygen saturation must be carefully monitored
to prevent hypercapnic respiratory failure.
TREATMENT
Emerging
Inhaled fluoroquinolone therapy
Both ciprofloxacin dry powder for inhalation and liposomal ciprofloxacin for inhalation reduced bacterial load
in randomised placebo-controlled phase II studies of patients with non-cystic fibrosis bronchiectasis who
were culture-positive for respiratory pathogens, including Pseudomonas .[67] [68] Phase III clinical trials to
investigate the potential benefits of inhaled fluoroquinolone therapies are under way.
Neutrophil elastase inhibitor

AZD9668 is a novel, orally active, reversible inhibitor of human neutrophil elastase that has been shown to
reduce neutrophil elastase activity by >90% in healthy controls and in COPD patients. AZD9668 at 60 mg
twice daily was studied in 38 adults with non-cystic fibrosis bronchiectasis in a randomised, double-blind,
placebo-controlled phase II trial, over a 28-day treatment period. The primary endpoints were neutrophil
count in waking and post-waking sputum samples, and lung function. The drug was well tolerated but there
was no statistically significant difference in sputum neutrophils or neutrophil elastase activity compared with
the control group. There was, however, a statistically significant decrease in sputum CCL5, a chemotactic
factor for T cells. The treatment group also had a small, and probably sub-clinical, but statistically significant
increase in FEV1. Significant inter- and intra-patient variability in outcome variables was seen; thus, larger
and longer-term studies will be important to confirm these initial findings.[69]
TREATMENT
31
Bronchiectasis Follow up
Recommendations
Monitoring
FOLLOW UP
Although chest CT scan is the diagnostic procedure of choice, CXR (PA and lateral) is sufficient for
subsequent monitoring. Surveillance sputum cultures are recommended on a regular basis to monitor for
changes in antibiotic sensitivities of organisms present in sputum. Spirometry should be measured with
most surgery visits and a reduction in the FEV1 or FEV1/FVC ratio is regarded as an indicator of disease
progression or exacerbation. Weight should be recorded during every visit.
Patient instructions
Airway clearance in bronchiectasis is paramount, so patients should be instructed to complete mucus
clearance techniques 2 to 3 times per day. A healthy, balanced diet is optimal for achieving a BMI above
18.5. After referral to pulmonary rehabilitation, patients are encouraged to continue a similar exercise
programme on a regular basis. It is recommended that patients contact their healthcare provider at the
first indication of exacerbation (fever, change in sputum colour or volume, or dyspnoea).
Complications
Complications Timeframe Likelihood

massive haemoptysis variable low
Massive, life-threatening haemoptysis (>250 mL/day) may occur.
Protection of the airway is achieved by reclining the patient on the side the bleeding is suspected. If
needed, endotracheal intubation is recommended.
Breathing and circulation are monitored and supported as needed. Immediate referral to a thoracic
surgeon and/or interventional radiologist is essential for bronchoscopy-guided haemostatic tamponade,
bronchial artery embolisation, or surgical resection of the involved area.[76] [77] Haemoptysis is thought to
originate from the bronchial arteries or bronchial-pulmonary anastomoses.
respiratory failure variable low
A primary goal is to prevent or treat tissue hypoxia.
Treatment includes airway protection, supplemental oxygen, potentially mechanical ventilation, and
treatment of the underlying cause.
cor pulmonale variable low
Early and effective treatment helps to prevent cor pulmonale. If there is evidence of pulmonary
hypertension, immediate referral to a pulmonologist and/or thoracic surgeon is optimal.
In the most severe cases, heart and lung transplantation may be necessary.
Bronchiectasis Follow up
Prognosis
FOLLOW UP
Bronchiectasis is an irreversible condition. The typical disease course consists of periods of symptom
control interrupted by periods of exacerbations. Bronchiectasis frequently co-exists with other respiratory
disease, making it difficult to determine prognosis for bronchiectasis alone.[70] Factors associated with a
faster decline in lung function include more frequent severe exacerbations.[71] Hypoxaemia, hypercapnia,
dyspnoea, and radiographic extent of disease have been shown to correlate with mortality. Conversely, a
higher BMI, regularly scheduled doctor visits, and vaccinations improve survival.[51]
A bronchiectasis severity index, validated in the UK and Europe, may help with understanding prognosis and
guiding treatment.[72] [73] [74]
Quality of life
According to the St. George Respiratory Questionnaire, dyspnoea, reduced FEV1, and daily sputum
production have the greatest impact on the quality of life in patients with bronchiectasis.[75]
Pseudomonas species in sputum

Pseudomonas species in the sputum of patients with bronchiectasis indicates more extensive lung disease
and more severe impairment of pulmonary function than in patients without Pseudomonas species
colonisation. Some studies have shown that Pseudomonas species colonisation is an independent factor
associated with a faster decline in lung function. Evidence is conflicting.[71]
33
Bronchiectasis Guidelines
Diagnostic guidelines
Europe
Guideline for non-CF bronchiectasis

Published by: British Thoracic Society Last published: 2010
BTS statement on criteria for specialist referral, admission, discharge and

follow-up for adults with respiratory disease
Recommendations for the assessment and management of cough in children

Recommendations for the management of cough in adults

GUIDELINES
North America
ACR appropriateness criteria: hemoptysis

Published by: American College of Radiology Last published: 2014
ACR appropriateness criteria: chronic dyspnea - suspected pulmonary origin

Published by: American College of Radiology Last published: 2012
Chronic cough due to bronchiectasis: ACCP evidence-based clinical practice

guidelines
Published by: American College of Chest Physicians Last published: 2006
Guidelines for evaluating chronic cough in pediatrics: ACCP evidence-based

clinical practice guidelines
Oceania
Chronic suppurative lung disease and bronchiectasis in children and adults

Published by: Thoracic Society of Australia and New Zealand; Last published: 2010
Australian Lung Foundation
Bronchiectasis Guidelines
Treatment guidelines
Europe
Bronchiectasis (non-cystic fibrosis), acute exacerbation: antimicrobial

prescribing
Published by: National Institute for Health and Care Excellence Last published: 2018
Guidelines for the management of adult lower respiratory tract infections

Published by: European Respiratory Society Last published: 2011
Guideline for non-CF bronchiectasis

BTS statement on criteria for specialist referral, admission, discharge and
GUIDELINES
follow-up for adults with respiratory disease
Recommendations for the assessment and management of cough in children

Recommendations for the management of cough in adults

North America
Chronic cough due to bronchiectasis: ACCP evidence-based clinical practice

guidelines
Oceania
Chronic suppurative lung disease and bronchiectasis in children and adults

Published by: Thoracic Society of Australia and New Zealand; Last published: 2010
Australian Lung Foundation
35
Bronchiectasis References
Key articles
• Reid LM. Reduction in bronchial subdivision in bronchiectasis. Thorax. 1950;5:233-247. Full text
REFERENCES
Abstract
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• Pasteur MC, Bilton D, Hill AT, et al. British Thoracic Society guideline for non-CF bronchiectasis.
Thorax. 2010;65(suppl 1):i1-i58. Full text Abstract
• Serisier DJ, Martin ML, McGuckin MA, et al. Effect of long-term, low-dose erythromycin on pulmonary
exacerbations among patients with non-cystic fibrosis bronchiectasis: the BLESS randomized
controlled trial. JAMA. 2013;309:1260-1267. Full text Abstract
• Chalmers JD, Smith MP, McHugh BJ, et al. Short- and long-term antibiotic treatment reduces
airway and systemic inflammation in non-cystic fibrosis bronchiectasis. Am J Respir Crit Care Med.
2012;186:657-665. Abstract
• Chalmers JD, Goeminne P, Aliberti S, et al. The bronchiectasis severity index. An international
derivation and validation study. Am J Respir Crit Care Med. 2014;189:576-585. Full text Abstract
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41
Bronchiectasis Images
Images
IMAGES
Figure 1: Mucus-impacted bronchi in a 36-year-old woman with allergic bronchopulmonary aspergillosis

From Pamela J. McShane, MD; used with permission
IMAGES
Figure 2: Situs inversus (Kartagener's syndrome) in a 19-year-old woman with focal bronchiectasis from
primary ciliary dyskinesia
43
IMAGES Bronchiectasis Images
Figure 3: CXR with dilated and thickened airways

From archives of Dr Sangeeta M. Bhorade; used with permission
IMAGES
Figure 4: CXR with lack of normal tapering producing a tram line
45
Figure 5: Chest CT with dilated and thickened airways and peripheral tree-in-bud pattern
IMAGES
Figure 6: Chest CT with presence of signet ring on left
47
Figure 7: Signet ring signs in a 20-year-old woman with bronchiectasis

IMAGES
Figure 8: Severe cystic and varicose bronchiectasis in a 49-year-old man with idiopathic bronchiectasis and
scoliosis
49
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Contributors:
// Authors:
Anne E. O'Donnell, MD
Professor of Medicine
Chief, Division of Pulmonary, Critical Care and Sleep Medicine, Georgetown University Hospital,
Washington, DC
DISCLOSURES: AEOD has been compensated by the following companies for consultation regarding
new drug development and clinical trials: Bayer, Xellia, Novartis, Insmed. AEOD has been or is the
Principal Investigator for trials sponsored by the following companies (research funding provided directly
to Georgetown University): Bayer, Insmed, Aradigm, Gilead. AEOD is the Principal Investigator for the
Bronchiectasis Research Registry, sponsored by the COPD Foundation (research funding provided directly
to Georgetown University). AEOD is the author of a reference cited in this monograph.
// Acknowledgements:
Dr Anne E. O'Donnell would like to gratefully acknowledge Dr Pamela J. McShane and Dr Sangeeta M.
Bhorade, previous contributors to this monograph. PJM and SMB declare that they have no competing
interests.
// Peer Reviewers:
Philip W. Ind, BA (Cantab), MB BChir, MA (Cantab), FRCP

Consultant Physician
Honorary Senior Lecturer, Imperial College Healthcare Trust, Hammersmith Hospital, London, UK
DISCLOSURES: PWI declares that he has no competing interests.
Ware Kuschner, MD
Associate Professor of Medicine
Stanford University, Stanford, Staff Physician, US Department of Veterans Affairs, Palo Alto Health Care
System, Palo Alto, CA
DISCLOSURES: WK declares that he has no competing interests.

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Bronchiectasis

The right clinical information, right where it's needed

Last updated: Mar 21, 2018

◊ Diagnostic testing for a potential aetiology of unexplained bronchiectasis should be performed.

◊ Daily airway clearance is essential for treatment success.

◊ Maintenance aerosolised antibiotics should be used for treatment of severe bronchiectasis or

• Pseudomonas aeruginosa infection

• Mounier-Kuhn’s syndrome (also known as tracheobronchomegaly, characterised by abnormal

• 7% to 50% of cases.[10] [12]

• Bronchi enlarged and cylindrical in shape

• Irregular bronchi, with alternating dilation and constriction

• Most severe form

Step-by-step diagnostic approach

• Age at presentation is over 40 years and no other identified cause

Serum alpha-1 antitrypsin phenotype and level

• Recommended to identify alpha-1 antitrypsin disease as underlying aetiology.

• Recommended to identify individual immunoglobulin deficiencies as underlying aetiology.

• Recommended in all patients.

• Recommended in all patients.

• Recommended in patients with a history of frequent childhood sinopulmonary infections to evaluate

congenital disorders of the bronchial airways

primary ciliary dyskinesia

connective tissue disease

inflammatory bowel disease

aspiration or inhalation injury

focal bronchial obstruction

tall, thin, white females, aged 60 or over

History & examination factors

sputum production (common)

crackles, high-pitched inspiratory squeaks and rhonchi (common)

• Often correlates with severity of bronchiectasis on chest CT.

Other diagnostic factors

weight loss (common)

pleuritic chest pain (uncommon)

rheumatoid factor positive rheumatoid factor

Other tests to consider

• Nasal or bronchial tissue is analysed by video microscopy to evaluate ciliary dyskinesia

Condition Differentiating signs / Differentiating tests

asthma • Inspiratory squeaks and • Chest CT may show

Condition Differentiating signs / Differentiating tests

chronic sinusitis • The inspiratory squeaks • CXR and chest CT

Step-by-step treatment approach

Maintenance therapy for all patients

Airway clearance includes:

• Maintenance of oral hydration.

erdosteine, and bromhexine.[47] [48]

• A healthy diet and exercise are recommended in all patients.

Maintenance therapy for select patients

Treatment of refractory cases

Long-term ox ygen therapy

• FEV1 <30% predicted or rapid decline in FEV

• Refractory or recurrent pneumothorax

Treatment details overview

1st exercise and improved nutrition

plus airway clearance therapy

adjunct inhaled bronchodilator

adjunct inhaled hyperosmolar agent

adjunct long-term oral macrolide

with high risk for plus inhaled antibiotic

with acute exacerbation: plus short-term oral antibiotic

with acute exacerbation: plus short-term intravenous antibiotic

with recurrent infections, adjunct surgery

with severe ventilatory plus non-invasive ventilation