AJTCCM VOL. 29 NO. 2 2023 71
GUIDELINES
Background. Bronchiectasis is a chronic lung disorder that aects the lives of many South Africans. Post-tuberculosis (TB) bronchiectasis is an
important complication of previous pulmonary TB and a common cause of bronchiectasis in South Africa (SA). No previous statements on the
management of bronchiectasis in SA have been published.
Objectives. To provide a position statement that will act as a template for the management of adult patients with bronchiectasis in SA.
Methods. e South African oracic Society appointed an editorial committee to compile a position statement on the management of adult
non-cystic brosis (CF) bronchiectasis in SA.
Results. A position statement addressing the management of non-CF bronchiectasis in adults in SA was compiled. is position statement covers
the epidemiology, aetiology, diagnosis, investigations and various aspects of management of adult patients with non-CF bronchiectasis in SA.
Conclusion. Bronchiectasis has largely been a neglected lung condition, but new research has improved the outlook for patients. Collaboration
between interprofessional team members in patient management is important. In SA, more research into the epidemiology of bronchiectasis,
especially post-TB bronchiectasis and HIV-associated bronchiectasis, is required.
Keywords. Bronchiectasis, non-cystic brosis, management.
Afr J Thoracic Crit Care Med 2023;29(2):e647. https://doi.org/10.7196/AJTCCM.2023.v29i2.647
Bronchiectasis is a heterogeneous disease with multiple causes
affecting the airways that results in irreversible dilatation of the
bronchi, and is clinically characterised by a chronic cough, sputum
production and frequent exacerbations.[1,2] Cole’s ‘vicious cycle
hypothesis’ (Fig.1) of an inciting infection resulting in damage
to the airways and impaired mucociliary clearance, leading to
persistent airway inflammation, mucus accumulation, recurrent
respiratory tract infection and further airway damage, is a well-
recognised model for explaining the underlying pathogenesis of
bronchiectasis.[3]
South African oracic Society position statement on the
management of non-cystic brosis bronchiectasis in adults: 2023
A Goolam Mahomed,1 MB BCh, FCP (SA), FCCP ; S D Maasdorp,2 MB ChB, MMed (Int Med), FCP (SA), Cert Pulmonology (SA) Phys;
R Barnes,3 BSc (Physio), MSc (Physio), PhD; H van Aswegen,4 PhD; A Lupton-Smith,5 BSc (Physio), PhD;
B Allwood,6 MB BCh, FCP (SA,) Cert Pulmonology (SA) Phys, MPH, PhD;
G Calligaro,7 BSc Hons, MB BCh, FCP (SA), MMed (Int Med), Cert Pulmonology (SA) Phys;
C Feldman,8 MB BCh, DSc, PhD, D Med (honoris causa), FRCP, FCP (SA);
I S Kalla,8 MB BCh, PhD, FCP (SA), FCCP, Cert Pulmonology (SA) Phys, Cert Critical Care (SA)
1 Louis Pasteur Private Hospital, Pretoria, South Africa
2 Division of Pulmonology and Critical Care, Department of Internal Medicine, Faculty of Health Sciences, University of the Free State, Bloemfontein,
South Africa
3 Department of Physiotherapy, Faculty of Health Sciences, University of the Free State, Bloemfontein, South Africa
4 Department of Physiotherapy, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa
5 Division of Physiotherapy, Department of Health and Rehabilitation Sciences, Stellenbosch University, Cape Town, South Africa
6 Division of Pulmonology, Department of Internal Medicine, Faculty of Medicine and Health Sciences, Stellenbosch University, Cape Town, South Africa
7 Division of Pulmonology, Department of Internal Medicine, Faculty of Health Sciences, University of Cape Town, South Africa
8 Department of Internal Medicine, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa
Corresponding author: A Goolam Mahomed (akhtergm@telkomsa.net)
e South African oracic Society mandated a multidisciplinary team of healthcare providers to compile a position statement on the
management of non-cystic brosis bronchiectasis in South Africa (SA). International guidelines on the management of bronchiectasis
were reviewed and used as a basis from which the current position statement was compiled. is is the rst position statement on the
management of adult non-cystic brosis bronchiectasis in SA. A description of the epidemiology and aetiology of bronchiectasis is
provided, as well as guidance on its diagnosis and management.
e position statement provides guidance on the management of bronchiectasis to healthcare providers, policymakers and regulatory
authorities.
72 AJTCCM VOL. 29 NO. 2 2023
GUIDELINES
More recently, Flume etal.[4] described the complex pathogenesis of
bronchiectasis as a ‘vortex’ whereby impaired airway mucociliary
clearance and secretion accumulation impair normal host immunity,
resulting in recurrent and/or persistent infection, which elicits a host
inammatory response that causes airway injury and remodelling,
ultimately leading to bronchiectasis.
Bronchiectasis aects the lives of many South Africans, but it is
a neglected condition, and very little local research is available for
review. No previous statements on the management of bronchiectasis
in South Africa (SA) have been published. Given that 2019 was the
200th anniversary of the rst description of bronchiectasis by Laënnec
in 1819, the Council of the South African oracic Society (SATS)
resolved to develop a consensus statement on the management
of adult non-cystic brosis (CF) bronchiectasis in SA to celebrate
this occasion. An editorial committee under the leadership of Prof.
Akhter Goolam Mahomed and Dr Shaun Maasdorp was appointed
to perform this task.
Our objective was to provide a position statement that will act as
a template for the management of adult patients with bronchiectasis
in SA. e position statement is aimed at all healthcare practitioners
including primary care practitioners (GPs), physician specialists,
pulmonologists, nurses, physiotherapists, surgeons, pharmacists, and
ancillary specialists such as microbiologists and radiologists. We hope
that it will serve as a stimulus to conduct local research in the eld
of bronchiectasis, as we have many unanswered questions, and we
would also like it to serve as a template for guidance to policymakers
and regulatory authorities. A separate consensus document on
the management of CF has been published by the South African
Cystic Fibrosis Association.[5] The management of CF-associated
bronchiectasis is discussed in detail in that document, and therefore
will not be covered in the current document.
Members of the editorial committee comprised a balance of
specialist representatives with an interest in bronchiectasis from the
various academic sectors and private practice in SA. Each expert
member of the editorial committee was tasked with writing a section
of the document, with particular reference to the content and
evidence grading of the latest version of the British oracic Society[6]
and European Respiratory Society[7] guidelines on the management
of bronchiectasis, as well as the SA consensus guideline on the
management of CF.[5] e members communicated with each other
via e-mail and telephonically. e nal dra document was compiled
by the editorial committee and circulated among SATS Council
members for further comment. ese comments were considered by
the editorial committee, and the nal dra was then completed aer a
nal face-to-face meeting of the committee at the 2022 SATS Congress
in Cape Town.
Position statement
1. Epidemiology
ere is marked regional variation in the prevalence of bronchiectasis.
In the USA, the prevalence of bronchiectasis was estimated to be 139 per
100000 among adult patients.[8] Bronchiectasis becomes more common
with increasing age. Among US Medicare patients aged ≥65years, the
estimated prevalence was 701 per 100 000.[9] Patients enrolled in the
US Bronchiectasis Research Registry were mostly female (79%), and
the mean age at diagnosis was 57 years.[10] A UK population-based
cohort study found the prevalence of bronchiectasis to be 566 per
100 000 in women and 485 per 100 000 in men.[11] Bronchiectasis was
similarly more common in women, and the median age at diagnosis
was 61.8years.[11] e age-adjusted mortality rate for women with
bronchiectasis was 1 437.7 per 100000, a rate that was 2.26 times
higher than that of the general population.[11] Similarly, among men,
the age-adjusted mortality for patients with bronchiectasis was 1914.6
per 100000, which was 2.14 times higher compared with the general
population.[11] InGermany, the prevalence of bronchiectasis was 67 per
100000, with a higher prevalence of 192 per 100000 in patients aged
>65 years.[12] Patients with bronchiectasis in India tend to be younger
(medianage 56 years) than European and US patients and are more
likely to be male (56.9%).[13] e highest prevalence of bronchiectasis
was reported from China, with 1.2% (1 200 per 100000) of individuals
aged >40 years being diagnosed with bronchiectasis.[14] However,
bronchiectasis is by no means a condition that only aects older people.
Recommendations
• In South Africa, the most common aetiology of bronchiectasis
is post-infectious, with tuberculosis being the most important
pathogen.
• A work-up for secondary causes of bronchiectasis should
be performed in all patients diagnosed with diffuse
bronchiectasis.
• e investigation of choice for the diagnosis of bronchiectasis
should be a thin-slice computed tomography scan of the chest
(if a chest radiograph is non-diagnostic).
• Sputum Gram stain and culture should be a routine procedure
in all cases of bronchiectasis and should be done initially,
during follow-up visits, and before initiation of antibiotics in
cases with exacerbations. is allows for targeted therapeutic
interventions.
• Airway clearance techniques remain the mainstay of therapy.
• Bronchiectasis can coexist with other respiratory and
systemic illnesses. ese need to be identied and treated
independently.
• All patients should receive an annual inuenza vaccination
and the pneumococcal vaccination, and should be vaccinated
against COVID-19. e dosing schedule should be determined
by the most current guidelines published.
Damage to airways/
mucociliary dysfunction
Mucus
accumulation
Airway infection
Airway
inammation
Fig.1. Pathogenesis of bronchiectasis.
AJTCCM VOL. 29 NO. 2 2023 73
GUIDELINES
Both idiopathic and post-tuberculosis (TB) bronchiectasis are found in
children and young adults.[15] Bronchiectasis is prevalent among patients
with HIV.[16,17] Although population-based studies are lacking in Africa,
it ishighlylikely that bronchiectasis is common and frequently under-
diagnosed given the high burden of infectious pulmonary diseases,
especially pulmonary TB and HIV, on this continent.
2. Aetiology
e list of conditions associated with bronchiectasis is extensive, as
indicated in Table1.[3,18] ere is marked geographical variation in the
aetiology of bronchiectasis.[15] In India, TB (35.5%) followed by other
infections (22.4%) are the main causes of bronchiectasis.[13] ese
gures contrast strikingly with an Australian bronchiectasis cohort,
where post-infective bronchiectasis (28.1%) was common, but post-
TB bronchiectasis was only found in 1.8%.[19] In a European cohort,
the most common causes of bronchiectasis included infections,
connective tissue disease, immunodeciency and asthma.[20] However,
idiopathic bronchiectasis still made up 40% of the study population,
and this is common to all registries, especially if an exhaustive
evaluation is not performed.[20]
2.1 Post-TB bronchiectasis
It is interesting to appreciate that, for the rst 150 of the 200 years
since it was rst described, bronchiectasis was strongly associated
with TB, yet until recently most modern publications from low-
TB settings fail to fully acknowledge TB as a major global cause
of non-CF bronchiectasis. It has recently been estimated that
155million TB survivors are currently alive,[21] with many of them
having some form of residual impairment.[22] e proportion of non-
CF cases attributable to TB naturally varies substantially according
to the population background incidence of TB, for example being
estimated at 12% in Taiwan[23] and 36% in the recent European
Multicentre Bronchiectasis Audit and Research Collaboration
(EMBARC) study in India.[13] Viewed the other way, the nding of
bronchiectasis aer TB also appears to vary widely, ranging from 4 -
11% on chest radiographs to 35 - 86% on the more sensitive computed
tomography (CT) scan imaging.[24] Recently, chest radiographic imaging
in a prospective Malawian cohort found bronchiectasis in ≥1 lobe in
44.2% of adult patients aer treatment completion.[25]
TB may result in damage to a number of dierent compartments of
the lung, namely the parenchyma, large and small airways, vessels and
pleura.[26] Post-TB bronchiectasis therefore forms one of a cluster of
possible manifestations involved in the umbrella term ‘post-TB lung
disease’ and may coexist with, among others, small-airways disease (or
TB-associated obstructive lung disease) and lung brosis in any one
individual patient.[26]
Post-TB bronchiectasis appears to have a male preponderance,
probably reecting the well- known male preponderance of TB,[13]
aects the upper lobes more commonly than other causes of non-CF
bronchiectasis, and is more likely to be unilateral.[23] However, the
anity for the middle and lower zones is oen under-appreciated,
being involved in ~60% of post-TB bronchiectasis cases.[13] Additionally,
post-TB bronchiectasis appears to be more frequently associated with
a ‘frequent exacerbator’ phenotype compared with other causes,[13] and
although most commonly associated with airow obstruction, is also
more frequently associated with low forced vital capacity compared with
other causes.[13]
Isolation of other organisms in post-TB bronchiectasis patients
is not uncommon, but appears to vary substantially by geographical
location, with common organisms including Pseudomonas aeruginosa,
non-tuberculous mycobacteria (NTM), Haemophilus inuenzae and
Klebsiella pneumoniae.[13,23,27]
Interestingly, there is emerging evidence suggesting that the
Bronchiectasis Severity Index (BSI) and FACED score (forced expiratory
volume in 1 second (FEV1), age, colonisation with P. aeruginosa, number
of pulmonary lobes aected, and dyspnoea) are unable to adequately
predict exacerbations and readmissions in post-TB bronchiectasis, but
do demonstrate an association with mortality.[28]
3. Diagnosis
The main symptom of bronchiectasis in adults is a chronic cough
productive of mucopurulent sputum,[18] although bronchiectasis can also
occur in the absence of sputum production. Associated symptoms such
as dyspnoea, weight loss, fatigue and haemoptysis may develop as the
disease progresses.[18] Any history of persistent mucopurulent sputum
production therefore raises the possibility of underlying bronchiectasis.
[6] However, in patients who are not immunocompromised, reactivation
adult TB occurs in the upper lobes, which in the upright position allows
for spontaneous drainage of mucus. ese patients therefore oen do
not present with a productive cough, and this has been referred to as
‘dry bronchiectasis’. A comprehensive clinical history and meticulous
clinical examination can reveal additional symptoms and signs
associated with an underlying cause of bronchiectasis, for example a
history of steatorrhoea with CF or the nding of situs inversus with
Kartagener’s syndrome.[29] e diagnosis of bronchiectasis can oen be
suspected on chest radiographic imaging, but thin-slice CT has superior
sensitivity and specicity and is therefore the imaging modality of choice
for conrmation of disease. Morphologically, bronchiectasis on CT
images can have a cylindrical, varicose or cystic appearance.[29] Current
Table1. Conditions associated with bronchiectasis[3,18]
Post-infectious
Mycobacterial infection, especially tuberculosis
Viral infection, such as measles, whooping cough, COVID-19
Pneumonia
Mucociliary disorders
Cystic brosis
Primary ciliary dyskinesia
Secondary to bacterial infection
Obstruction
Foreign body, tumour, mycobacterial infection
Immune dysfunction
Primary: Hypogammaglobulinaemia, selective
hyperglobulinaemia, neutrophil abnormalities
Secondary: HIV, chemotherapy, allergic bronchopulmonary
aspergillosis, malnutrition, extremes of age
Rheumatic/inammatory conditions
Rheumatoid arthritis, inammatory bowel disease
Miscellaneous
Congenital tracheobronchomegaly, Marfan’s syndrome,
alpha-1-antiprotease deciency syndrome, chronic
obstructive pulmonary disease, asthma, aspiration
74 AJTCCM VOL. 29 NO. 2 2023
GUIDELINES
criteria for diagnosing bronchiectasis on CT
imaging include:
• a broncho-arterial ratio (internal
diameter of bronchus/outer diameter
of accompanying pulmonary artery)
>1 (‘signet-ring sign’)
• absence of normal airway tapering
towards the lung periphery (‘tram
track’ appearance)
• visible small bronchi within 1 cm from
the costal visceral pleura or abutting
mediastinal pleura
• thickened bronchial walls
• thick secretions in airways
• mosaic attenuation on expiratory CT
views.[6,30,31]
The fixed cut-off value for the broncho-
arterial ratio has recently been contested,
because age- and disease-related changes
in both the airway and vessel diameter can
occur that may decrease the sensitivity and
specicity of this measurement.[32] Magnetic
resonance imaging of the chest, by which
both structural and functional aspects of the
lungs can be measured, is rapidly developing
as an imaging modality for respiratory
diseases and may play an important role in
the future management of bronchiectasis.[32]
4. Management
The main principles of treating patients
with bronchiectasis are to identify and treat
the underlying cause, as well as optimally
managing secretions (oen mucopurulent),
recurrent airway infections and persistent
airway inflammation that can lead to
worsening lung function over time (Fig.2).
Exacerbations of bronchiectasis contribute
significantly to morbidity, mortality and
healthcare costs and are therefore a major
target for prevention, early recognition and
expedited treatment. An exacerbation of
bronchiectasis is dened as deterioration in
≥3of the symptoms of coughing, sputum
volume and/or consistency, sputum
purulence, dyspnoea and/or exercise
tolerance, fatigue and/or malaise, and
haemoptysis over at least a 48-hour period, in
addition to a clinician’s decision that a change
in treatment for bronchiectasis is required.[33] e
frequent-exacerbator phenotype, dened as
≥3 exacerbations per year, carries the highest
risk for recurrent exacerbations, reduced
quality of life and increased mortality over a
5-year period.[34]
4.1 Airway clearance
Airway clearance techniques (ACTs) are
non-pharmacological interventions used to
facilitate removal of retained secretions from
the airways of patients with chronic respiratory
diseases such as bronchiectasis.[35] Short-term
goals for the managementofpatients living
with bronchiectasis using ACTs include
more effective removal of retained and
infected secretions to relieve symptoms of
Diagnosis
• Symptoms
• Chest X-ray
• HRCT (focal or diuse)
Aetiology
• Focal • Diuse
• Post-infectious • Primary ciliary diseases
• TB/NTM/bacteria • Immune dysfunction disorders
• Childhood infections/viral • Connective tissue diseases
• Aspiration
• Obstruction
Treat the cause
• For example, immunoglobulin replacement therapy for immunoglobulin deciency syndromes
Airway clearance techniques
• Active cycle of breathing Oscillating positive expiratory pressure devices
• Hung (direct hung) Bronchodilator
• Postural drainage Mucolytics – isotonic or hypertonic saline nebulisation
• Dyspnoea management Health dialogue (education)
• Physical exercise Self-management
Management – phenotype specic
• Primary
• Sputum culture-directed therapy
• Secondary
• Targeted therapy to underlying disorder
• Sputum culture-directed therapy
• Frequent exacerbators
• Long-term therapy – macrolides or inhaled antibiotics
• Sputum culture-directed therapy
Vaccination
• Annual inuenza vaccination
• Pneumococcal vaccination
• COVID-19 vaccination
Additional
• Nutritional support Risk stratication
• Diuretics for cor pulmonale • FACED score
• Lung transplant • BSI score
Follow-up
• Multidisciplinary team Dedicated bronchiectasis clinics
• Physiotherapist Work-up for lung transplant
• Microbiologist Management of underlying comorbidities
• Pulmonologist
• Dietician
•Symptoms
•Chest X-ray
•HRCT (focal or diffuse)
Diagnosis
•Focal
•Diffuse
•Post-infectious Primary ciliary diseases
•TB / NTM / Bacterial Immune dysfunction disorders
•Childhood infections / Viral Connective tissue diseases
•Aspiration
•Obstruction
Aetiology
•For example immunoglobulin replacement therapy for immunoglobulin deficiency syndromes
Treat the cause
•Active cycle of breathing Oscillating positive expiratory pressure devices
•Huffing (direct huffing) Bronchodilator
•Postural drainage Mucolytics -isotonic or hypertonic saline nebulisation
•Dyspnoea management Health dialogue (education)
•Physical exercise Self-management
Airway clearance
•Primary
•Sputum culture-directed therapy
•Secondary
•Targeted therapy to underlying disorder
•Sputum culture-directed therapy
•Frequent Excerbators
•Long-term therapy -macrolides or inhaled antibiotics
•Sputum culture-directed therapy
Management - Phenotype specific
•Annual influenzae vaccination
•Pneumococcal vaccination
•COVID-19 vaccination
Vaccination
•Nutritional support Risk stratification
•Diuretics for cor pulmonale FACED score
•Lung transplantation BSI score
Additional
•Multi-disciplinary team Dedicated Bronchiectasis Clinics
•Physiotherapist Work-up for lung transplant
•Microbiologist Management of underlying co-morbidities
•Pulmonologist
•Dietician
Follow-up
Fig. 2. Bronchiectasis management. (HRCT = high-resolution computed tomography;
TB=tuberculosis; NTM = non-tuberculous mycobacteria; FACED = forced expiratory volume in
1second, age, colonisation with Pseudomonas aeruginosa, numbers of pulmonary lobes aected
and dyspnoea; BSI = Bronchiectasis Severity Index.)
AJTCCM VOL. 29 NO. 2 2023 75
GUIDELINES
dyspnoea and to improve lung ventilation. Long-term goals for patient
management using ACTs include minimising the risk of further
airway damage, improving exercise capacity to optimise functional
independence, reducing the number of disease exacerbations and
hospitalisations per year, and improving disease-specic quality of
life (QoL) and cough-related QoL.[6,7] ACTs consist of a variety of
strategies used by physiotherapists in their management of patients
living with bronchiectasis during the acute exacerbation stage and
the chronic stage of their disease.[36] Physiotherapists are essential in
assistingand training patients with regard to the most appropriate
methods of airway clearance. CT imaging, if available, should be
reviewed to complement physiotherapy patient management by
identifying the aected lung segments.[6] e choice of ACT used
depends on preference, the age and level of comprehension of each
individual patient, and the presence or absence of contraindications
to the use of each ACT.[36] e chosen ACT should form part of the
patient’s daily routine. e frequency and duration of airway clearance
sessions should be tailored to the individual patient’s requirements
and may alter during periods of exacerbation.[6] Typically, treatment
is performed twice a day, for a minimum of 10 minutes to a maximum
of 30 minutes per session; however, during an exacerbation, treatment
frequency and duration can be adapted as required depending on each
individual patient’s clinical presentation and needs.[6]
e ACTs perceived to be most eective during acute exacerbation
and the stable stage of the disease include active cycle of breathing
technique, directed hung or forced expiratory technique, gravity-
assisted body positioning (postural drainage), modified body
positioning for postural drainage (no head-down tilt), oscillating
positive expiratory pressure (PEP) devices, continuous PEP therapy
(PEP mask), and exercise therapy.[16,34-36] Manual chest physiotherapy
techniques such as percussion, shaking and vibration combined with
gravity-assisted positioning or modied postural drainage may be used
for patients who struggle to expectorate their secretions when using
other ACTs.[6] A lesser-known but eective and inexpensive ACT is
slow expiration with the glottis held open in a lateral decubitus position
(ELTGOL – l’Expiration Lente Totale Glotte Ouverte en decubitus
Latéral) and is worth considering in the management of these patients,
in isolation or in combination with other ACTs.[37]
Muco-active therapy should be considered if ACTs are not eective
and should be co-ordinated with ACTs to ensure that the overall eect
is optimal.[38] e use of mucolytic therapy in patients with non-CF
bronchiectasis has not been adequately studied. It is therefore frequently
advised to use similar guidelines as for patients with CF, where isotonic
saline or hypertonic saline nebulisation therapy is recommended as
mucolytic agent. Hypertonic saline (7%) has an immediate eect, and
ACTs should therefore be used during or directly aer administration
of this drug.[38] If hypertonic saline does not seem to enhance secretion
clearance aer 4 weeks of use, it should be stopped. Other mucolytic
agents such as carbocysteine and erdosteine can be considered, although
evidence regarding ecacy is limited.[38] Humidication with sterile
water can also be considered.
In patients with features of reactive airway disease or asthma, or
severe airow obstruction (FEV1 <1 L/min), consider pretreatment
with bronchodilator therapy before administration of muco-active
therapy and ACTs. Short-acting beta-2-agonists such as salbutamol
via a metered dose inhaler or nebuliser device are recommended.
4.2 Pulmonary rehabilitation
Pulmonary rehabilitation is dened as ‘a comprehensive intervention
based on a thorough patient assessment followed by patient-tailored
therapies that include, but are not limited to, exercise training,
education, and behavior change, designed to improve the physical
and psychological condition of people with chronic respiratory
disease and to promote the long-term adherence to health-enhancing
b e h a v i o r s ’. [39] Patients with bronchiectasis frequently present with
reduced exercise capacity, muscle weakness and low levels of physical
activity, all of which contribute to worsening dyspnoea.[40] Pulmonary
rehabilitation should be oered to patients living with bronchiectasis
who suer from shortness of breath (Modied Medical Research
Council Dyspnoea Scale ≥1) that limits their physical functioning
and those with reduced exercise capacity.[6,40] Exercise consists of
a variety of activities, such as aerobic (cardiovascular) training
and resistance (strength) training. Exercise prescription should
follow a comprehensive assessment,[40,41] be individually tailored,
and follow the principles used for chronic obstructive pulmonary
disease (COPD). Inspiratory muscle training should be considered
in conjunction with exercise training to optimise training eects.[42]
Education sessions, tailored to the needs of individuals living with
bronchiectasis, form an essential part of pulmonary rehabilitation
programmes.[6,7,38] e benets of pulmonary rehabilitation include
improvements in exercise capacity, cough symptoms and QoL, and
in some cases reduced episodes of exacerbation.[43] ese benets are
usually obtained within 6 - 8 weeks of attendance and maintained
up to 6 months following completion of pulmonary rehabilitation.[7]
Maintenance programmes should be considered for patients upon
pulmonary rehabilitation programme completion. Patients who are
scheduled for surgical intervention for their bronchiectasis should
receive preoperative pulmonary rehabilitation to ensure that optimal
physical conditioning is achieved prior to surgery.[6,7]
4.3 Health dialogue (education) and self-management
Education should be provided to each individual living with
bronchiectasis, and the sessions should be tailored to the needs of
such individuals. Dialogue sessions should include explaining the
pathophysiology of the condition to the individual, identifying
appropriate ACTs to use for secretion management, management
strategies for dyspnoea during episodes of disease exacerbation,
the importance of physical exercise and hydration, and relevant
inhalation therapy.[6]
4.4 Exacerbations of bronchiectasis
Acute exacerbations of bronchiectasis are characterised by acute
bacterial infection, although viral infection may precede and trigger
this. An exacerbation of bronchiectasis is dened as a change in ≥3
of the following symptoms for >48 hours: cough, change in sputum
volume and/or consistency, sputum purulence, haemoptysis,
shortness of breath, and fatigue and/or malaise. Antibiotic therapy
is the cornerstone of treatment and reduces sputum bacterial load
as well as airway and systemic inammation.[44] erapy should
be tailored to previous sputum bacteriology results (if known), a
history of success or failure of prior regimens, and the presence of
allergy to antibiotics. Sputum should be routinely obtained for Gram
staining and culture prior to antibiotic administration. In addition
76 AJTCCM VOL. 29 NO. 2 2023
GUIDELINES
to excluding Mycobacterium tuberculosis, sputum should also be sent
for NTM culture. Patients may have received multiple courses of
antibiotics in the past, which increases the likelihood of resistant
organisms. A chest radiograph may be obtained if pneumothorax or
pneumonia is suspected.
Frequently isolated pathogens in bronchiectasis include
H.inuenzae,Moraxella catarrhalis,Staphylococcus aureus (methicillin
sensitive and resistant),P. aeruginosa(especially mucoid types), and,
less frequently,Streptococcus pneumoniae.[45,46] Afebrile and/or stable
patients can usually be treated with an oral antibiotic, while intravenous
treatment is reserved for patients with a clinical need for hospital
admission, failure of oral therapy, or pathogens that are resistant to
available oral agents. e choice of agent should be made according
to whether the colonising organism is sensitive or resistant to a beta-
lactam, and whether P. aeruginosa has been cultured. e respiratory
uoroquinolones (moxioxacin, levooxacin) are reasonable empirical
and broad-spectrum options if the sputum bacteriology is unknown.
In patients known to have P. aeruginosa airway infection and with
no quinolone resistance, ciprooxacin (500 - 750 mg twice a day) is
recommended.
Use of a single nebulised antibiotic for an infective exacerbation is
not recommended.
Clinical severity, treatment failure or conrmed resistance requires
the administration of intravenous antibiotics. In patients with
P.aeruginosa infection, the question of whether to use combination
antibiotics is controversial, and has not been well studied; a meta-
analysis of this practice in CF bronchiectasis was inconclusive.[47]
A common practice is to start with a single agent (for example, an
antipseudomonal beta-lactam cephalosporin or carbapenem) and to
add an aminoglycoside if the patient is acutely ill and pseudomonal
pneumonia seems possible. If methicillin-resistant S. aureus (MRSA) is
isolated, the patient can be treated with oral linezolid, co-trimoxazole
(if sensitive) or intravenous vancomycin.
e optimal duration of therapy is not well dened but is usually
10-14 days. ere are no good-quality data comparing shorter v.
longer regimens, and the recommendation of 14 days of antibiotics
in other international guidelines is based on expert consensus only.[7]
The isolation of P. aeruginosa is an important prognostic and
therapeutic consideration in patients with bronchiectasis, as it is
associated with a decline in lung function and an increased frequency
of exacerbations, hospital admissions and deaths.[48,49] Eradication
of P.aeruginosa cultured for the rst time is an attractive treatment
target (Table2); however, data on this therapy are limited, and studies
are small. In one controlled trial, 35 patients with new P. aeruginosa
were randomised to ceazidime/tobramycin intravenously followed
by three months of nebulised tobramycin 300 mg; 12 months later,
54% of the eradication group and 29% of the placebo group were free
of P.aeruginosa in sputum.[50]
4.5 Stable bronchiectasis
Daily oral non-macrolide antibiotic therapy (for example,
amoxicillin 500 mg twice daily and doxycycline 100 mg twice
daily) for bronchiectasis has not been well studied and is reserved
for patientswith ≥3 exacerbations per year who are not candidates
for long-term macrolide administration and are not colonised
with P. aeruginosa.[51] Patients with chronic airway infection
with P. aeruginosa can try inhaled antibiotic therapy. is is an
attractive strategy for chronic suppression, as there is negligible
systemic absorption and the antibiotic reaches exponentially higher
concentrations in the airways than if given intravenously. Inhaled
antibiotic options for patients with chronic P. aeruginosa infection
include gentamicin, tobramycin, amikacin and colistin. Inhaled
antibiotics should be preceded by administration of short- or long-
acting bronchodilator therapy followed by muco-active therapy and
ACTs.[7] Intermittent intravenous antibiotics are not part of routine
care outside of acute exacerbations, but may be given to patients
with resistant organisms before thoracic or non-thoracic surgery
to reduce bacterial burden and sputum production and optimise
postoperative lung function.
4.6 Anti-inammatory treatment
Patients with bronchiectasis who have recurrent exacerbations
(≥3per year) and are not colonised with P. aeruginosa should be
oered long-term anti-inammatory therapy with a macrolide.[7]
However, prior to the introduction of macrolide therapy it is important
to exclude the possibility that the cause of the bronchiectasis is
NTM, as use of a single-agent macrolide may be associated with
the development of drug-resistant NTM infection, especially
Mycobacterium avium-intracellulare, the treatment of which is
primarily macrolide based. Inpatients with chronic P. aeruginosa,
macrolides can also be added if they continue to have exacerbations
despite inhaled antipseudomonal antibiotic therapy. Macrolides are
antibiotics with anti-inammatory and immunomodulatory eects
that have been proven to signicantly reduce exacerbation frequency
in patients with bronchiectasis. eir use was extrapolated from the
CF literature, but several randomised controlled trials in patients
with non-CF bronchiectasis have conrmed their benet in terms of
reducing exacerbation frequency.[51-53] Adverse eects of macrolide
therapy include QT-interval prolongation, hearing loss and increased
bacterial resistance (Table3).[1] Inhaled corticosteroid therapy
should not be routinely provided to patients with bronchiectasis,
unless to treat a concomitant condition for which it is specically
indicated (e.g. asthma or COPD): a meta-analysis found insucient
evidence of benet.[53] A trial of therapy is therefore advised only
in adults with dicult-to-control symptoms or marked bronchial
hyper-responsiveness.
4.7 Role of surgery
Since bronchiectasis is associated with a decline in lung function over
time, a conservative approach to lung resection surgery is advised to
preserve functional lung parenchyma as much as possible. In patients
with localised bronchiectasis and recurrent infections, haemoptysis
and/or mycetoma in the affected part of the lung, or localised
infection with resistant organisms such as NTM or multidrug-
resistant or extensively drug-resistant TB, lung resection surgery
Table2. Pseudomonas aeruginosa eradication
P. aeruginosa cultured
Ciprooxacin 500 - 750 mg twice a day × 14 days followed by
inhaled colistin, gentamicin or tobramycin × 3 months
Intravenous antipseudomonal beta-lactam ± aminoglycoside
×14 days, followed by inhaled colistin, gentamicin or
tobramycin × 3 months
AJTCCM VOL. 29 NO. 2 2023 77
GUIDELINES
can be considered.[54,55] Patients with diuse bronchiectasis would
probably not benet from lung resection surgery, so medical therapy
remains the mainstay of treatment. Bilateral lung transplantation is an
option in selected patients with diuse bronchiectasis, and referral to
a specialised transplant centre should be considered.[56]
4.8 Causes of bronchiectasis with specic treatment
A rational approach to establishing an aetiological diagnosis is
required. The justification for investigating a secondary aetiology
is that in a few conditions, specific therapeutic interventions have
potential benets with minimal adverse eects. e conditions where
interventions with a specic aetiology may have a signicant impact
on outcome include immunoglobulin deciency syndromes, allergic
bronchopulmonary aspergillosis (ABPA) and NTM infections. e
recommended therapy for these conditions includes intravenous or
subcutaneous immunoglobulins for patients with defective antibody
production, oral corticosteroids with antifungal agents for ABPA,
and specic antimicrobial agents if the diagnosis of NTM has been
conrmed microbiologically. e approach as described in the British
oracic Society guideline for the management of bronchiectasis[6] is
endorsed by the SATS. In general, patients with diuse bronchiectasis
would require exclusion of conditions associated with a generalised
impairment of airway mucosa function such as CF or primary ciliary
dyskinesia, or a generalised immune dysfunction disorder such as
immunoglobulin deciency or HIV. A comprehensive clinical history
and examination should guide the clinician in the extent of the
diagnostic work-up. Routine investigations would usually include a
full blood count, urea, creatinine and electrolyte prole, liver function
tests and glucose determination. Assessment for underlying common
immune dysfunction disorders includes HIV testing, IgG, IgM and IgA
evaluation, as well as IgG subclasses and pneumococcal antibody testing.
Sweat chloride or conductivity testing (see the SA consensus guideline
on the management of CF[5]) should be included in the diagnostic work-
up of patients with diuse bronchiectasis or sinobronchitis. In patients
with features of asthma, screening for ABPA should be performed by
means of total IgE, Aspergillus-specic IgE or Aspergillus precipitants.
Sputum cultures, including mycobacterial cultures, are required in
all patients with bronchiectasis. Oen an underlying cause cannot be
identied, and referral to an immunologist may be required.
4.9 Immunisations
It is advisable that all patients with underlying chronic respiratory
conditions receive vaccinations. All adults should be oered annual
influenza vaccination with the trivalent/quadrivalent inactivated
influenza vaccine.[57,58] Patients with bronchiectasis who have not
previously received a pneumococcal conjugate vaccine or whose
previous vaccination history is unknown should receive a pneumococcal
conjugate vaccine (PCV13) followed by a dose of pneumococcal
polysaccharide vaccine (PPSV23).[59,60] e ecacy of pneumococcal
vaccination may be reduced in patients with immunoglobulin
deciency syndromes. Antibody titres to pneumococcal antigens may
be required to conrm an adequate immune response. An inadequate
antibody response would necessitate repeat pneumococcal vaccination.
Guidance on the timing intervals and dosing schedule are published in
the SA pneumonia guideline.[57]
Patients with bronchiectasis should also receive the COVID-19
vaccine. Data on the ecacy of all COVID-19 vaccines in this population
group, especially patients with combined variable immunodeciency
syndrome (CVID), are limited. e only data available for a similar
population group are from patients on immunosuppressive therapy
for solid-organ transplants. In this cohort of patients, the vaccine is
recommended even with the probability of reduced humoral ecacy,
as these patients have preserved cell-mediated immunity. Patients with
CVID should be immunised with two doses of COVID-19 vaccine.
[61,62] Literature to support an additional COVID-19 vaccine dose to
CVID patients is limited. However, data extrapolated from solid-organ
transplantation recipients undergoing immunosuppressive treatment
suggest that there is merit in receiving booster doses 9 months aer
receiving the last dose to counteract waning immunity.[61,62]
5. Prognosis
Age-adjusted mortality rates in patients with bronchiectasis were
double that of the general population in a UK population-based
cohort study.[11] Factors associated with a worse prognosis include
older age and comorbidities, lower FEV1, lower body mass index,
recurrent exacerbations, P. aeruginosa colonisation, a greater extent
of disease and worsening dyspnoea.[63,64] Several scoring systems,
such as the BSI,[63] accessible by means of an online calculator (http://
www.bronchiectasisseverity.com/15-2/), the Bronchiectasis Aetiology
Table3. Dierences between inhaled and oral macrolide antibiotics
Inhaled antibiotics Macrolide antibiotics
Advantages
High concentrations delivered to airways
Lower rate of resistance
Lower collateral damage
Low systemic absorption
Advantages
Easy to use, low treatment burden and relatively cheap
Reduce exacerbations
Anti-inammatory eects
Disadvantages
Concern over tolerance with nebulised formulation
Deposition erratic
Time consuming
Rotating regimens
Limited data in patients without Pseudomonas aeruginosa
Disadvantages
Gastrointestinal and other side-eects
Antibiotic resistance
Non-tuberculous mycobacteria colonisation must be excluded
Cardiovascular events/drug interactions (Q-Tc prolongation)
Long-term ecacy and tolerance
78 AJTCCM VOL. 29 NO. 2 2023
GUIDELINES
Comorbidity Index (BACI),[65] FACED[64] and
E-FACED (incorporating exacerbations),[66]
have been developed for predicting severity
and prognosis in patients with non-CF
bronchiectasis.
6. Follow-up
Bronchiectasis is a chronic condition with
variable rates of lung function decline,
dependent on colonisation with pathogenic
bacteria, especially Pseudomonas and
S. aureus, exacerbation frequency, and
adherence to airway clearance therapy.
Patients should therefore be followed up
frequently, at least 3 - 6-monthly, for clinical
review. Apart from the general history and
physical examination, adherence to ACT
schedules, as well as spirometry, should
form part of routine assessment at each visit.
Sputum for microscopy, culture and sensitivity
should be assessed 3-monthly (if possible),
and this should include culture for TB to
exclude infection with NTM. For patients
on long-term treatment with macrolides,
hearing should be assessed annually, and
macrolides should be discontinued if any
deterioration in hearing is found, while
also referring the patient appropriately for
audiology assessment.
7. Conclusion
An algorithmic approach to bronchiectasis
is provided in Fig. 3. Bronchiectasis has
largely been a neglected lung condition, but
the establishment of the EMBARC database
and recent new research into the condition
have improved the outlook for patients. We
have made progress in research into ACTs
and some progress in the use of appropriate
antibiotics for this condition. e importance
of collaboration among interprofessional
team members in patient management, with
clear communication regarding management
goals, collective planning for hospital
discharge and follow-up in the community
to ensure optimal clinical outcomes, cannot
be over-emphasised. However, several
questions remain unanswered. In SA we have
the opportunity to conduct research into the
epidemiology of bronchiectasis, post-TB
bronchiectasis, which seems to be a distinct
entity, and HIV-associated bronchiectasis.
Endorsement. The position statement has been
endorsed and approved by the Council of the SATS.
Declaration. AG-M, SDM, BA, GC, CF, and IK
are members of the editorial board.
Funding. All the work done by the writing
committee was voluntary. Some communication
costs and the transport and venue costs for the
nal meeting for some members were funded
by SATS.
Conicts of interest. None.
Disclaimer. This position statement is only
intended for guidance on general management
of patients. Each patient must be individually
managed. e nal responsibility for management
of any patient rests on the managing physician
and healthcare provider.
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Request chest X-ray
Chest x-ray in keeping
with bronchiectasis?
No
Request thin-slice CT chest
or high-resolution CT
Consider alternative
diagnosis
Airway clearance
techniques
Yes
If underlying cause
is identied,
treat specically
Vaccination:
Inuenza,
pneumococcal,
SARS-CoV-2
Antibiotics
General management
of bronchiectasis
Other: Nutrition,
diuretics,
lung transplant
Risk factors, symptoms and/or signs suggestive of bronchiectasis?
CT chest in keeping
with bronchiectasis?
No
Bronchiectasis conrmed
Routine tests: FBC, U&E and creatinine,
LFTs, glucose, HIV, sputum MCS
and TB culture
Further tests guided by clinical history
and ndings on examination: IgG, IgM,
IgA, IgG subclasses, total IgE,
Aspergillus IgE, sweat chloride
or conductivity, nasal or bronchial
mucosa biopsy
Fig.3. Algorithmic approach to bronchiectasis. (CT = computed tomography; FBC = full blood
count; U&E = urea and electrolytes; LFTs = liver function tests; MCS = microscopy, culture
and sensitivity; TB = tuberculosis; IgG = immunoglobulin G; IgM = immunoglobulin M;
IgA=immunoglobulin A; IgE = immunoglobulin E.)
AJTCCM VOL. 29 NO. 2 2023 79
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Submitted 12 December 2022. Accepted 10 May 2023. Published July 2023.
