BRONCHIECTASIS Flashcards
What is bronchiectasis?
Persistent or progressive chronic debilitating disease characterized by permanent dilation of the bronchi due to irreversible damage to the elastic and muscular components of the bronchial wall.
It can be focal (limited to one area of the lung) or diffuse
How does bronchiectasis present?
Symptoms can vary from intermittent expectoration and infection, to persistent daily expectoration of large volumes of purulent sputum.
Dyspnoea
Fever
Fatigue, reduced exercise tolerance
Haemoptysis
Rhinosinusitis
Weight loss
Chest pain
What are signs of bronchiectasis?
abnormal chest auscultation- coarse crackles and wheeze, high-pitched inspiratory squeaks
Palpable chest secretions on coughing or forced expiratory manoeuvre
clubbing may be present
What can cause bronchiectasis?
Idiopathic up to 40% of cases
1 in 3 cases are associated with a severe lung infection in childhood e.g. pneumonia, whooping cough, TB, measles
Immunodeficiency - IgA deficiency, IgG deficiency, , hypogammaglobulinaemia, HIV, malignancy
Allergic bronchopulmonary aspergillosis
Aspiration
CF
Cilia abnormalities e.g. primary ciliary dyskinesia, youngs syndrome
Connective tissue diseases - RA, Sjögren’s syndrome, Crohn’s, UC
Most commonly caused by a previous severe LRTI e.g. pneumonia or TB
post-infective: tuberculosis, measles, pertussis, pneumonia
bronchial obstruction e.g. lung cancer/foreign body/mucus plugging/lymph nodes
Mucociliary clearance dysfunction - primary ciliary dyskinesia, young syndrome, CF
Congenital airway defects - Williams-Campbell syndrome, mounier-Kuhn syndrome, youngs syndrome, Marfan syndrome
Others: allergic bronchopulmonary aspergillosis (ABPA), rheumatic diseases, COPD, IBD, yellow nail syndrome
Whats the epidemiology of bronchiectasis?
More common in females than males
60% of cases diagnosed over the age of 70 (>1% of over 70s have it)
Up to 25% of those with COPD might actually have bronchiectasis
Whats the pathophysiology of bronchiectasis?
Infective insults -> bronchial dilatation and mucociliary impairment -> infections and inflammation -> ongoing inflammatory cycle and further infections -> permenant airway remodelling which is dilatation of the airways with excessive mucous production
What are the most frequent pathogens found in bronchiectasis?
Haemophilus influenzae (20–40%)
Pseudomonas aeruginosa (10–30%),
Moraxella catarrhalis
Streptococcus pneumoniae, Staphylococcus aureus
Enterobacteriaceae
Non-tuberculous mycobacteria (NTM) accounts for 1–10% of cases.
Whats the prognosis of bronchiectasis?
People with mild bronchiectasis can have a normal life expectancy
Worse prognosis if extensive disease, have frequent severe exacerbations, dyspnoea, primary antibody deficiency disorder, colonisation with pseudomonas, comorbidity, smokers
Mortality rates are 2 fold higher than general population
Whats the strongest predictor of mortality in bronchiectasis?
Dyspnoea
Pseudomonas colonisation
What are complications of bronchiectasis?
Infective exacerbations and chronic bacterial colonisation
Haemoptysis
Pneumothorax
Respirator failure
Cor pulmonary
Chest pain
IHD
Anxiety and depression
Urinary incontinence
Nutritional deficiency
Fatigue and reduced exercise tolerance
Reduced QOL
When should you suspect an infective exacerbation of bronchiectasis?
There is a change in one or more of the common symptoms: increased sputum volume or purulence, worsening dyspnoea, increased cough, increased fatigue/malaise.
New symptoms develop, such as fever, pleuritic pain, or haemoptysis
How is bronchiectasis diagnosed?
CXR - may be normal or show non-specific abnormalities e.g. tram-track airways and ring shadows
Thin section CT - shows dilated bronchi with signet ring sign
What is the signet ring sign?
seen in bronchiectasis when the dilated bronchus and accompanying pulmonary artery branch are seen in cross-section. The bronchus and artery should be the same size, whereas in bronchiectasis, the bronchus is markedly dilated.
What investigations should be done for bronchiectasis?
Sputum culture
CXR
Spirometry
Oxygen sats
FBC
Others dependant on probable underlyign cause e.g. testing for primary ciliary dyskinesia, CF testing, serum total immunoglobulin etc
High resolution CT (thin section scanning)
How do you exclude allergic bronchopulmonary aspergillosis as a DDx for bronchiectasis?
Test serum total immunoglobulin - IgE and skin prick test to aspergillosis
Which pt with bronchiectasis should be followed up in secondary care?
Anyone at high risk of future exacerbations e.g.
had 3 or more exacerbations in a year
Chronic Pseudomonas aeruginosa, MRSA, or non-tuberculous mycobacteria colonization
Deteriorating bronchiectasis with declining lung function or advanced disease
Allergic pulmonary aspergillosis
Long term antibiotic therapy
Associated RA, immune defieicny, IBD, allergic bronchopulmonary aspergillosis, primary ciliary dyskinesia
Advanced disease and those considering lung transplantation
What tool can be used to determine the mortality and exacerbation rates in bronchiectasis?
Bronchiectasis severity index
(Takes into account age, BMI, FEV1%, previous hopsital admission, number of exacerbations in pervious year, MRC breathlessness score, pseudomonas colonisation, colonisation with others organisms, radiological severity)
What should be included in the annual review for bronchiectasis?
An assessment of symptoms and how they affect their daily lives — offer pulmonary rehabilitation to people with breathlessness that affects their daily lives.
Measurement of BMI — if the person’s BMI is less than 20 seek specialist nutritional advice.
The exacerbation frequency — refer people with three or more infective exacerbations a year, and people with fewer exacerbations that are causing significant morbidity to a respiratory specialist. May be considered for long-term prophylactic antibiotic treatment.
Taking a sputum sample for bacterial culture — specialist follow up is required for people with chronic colonization with Pseudomonas aeruginosa, opportunist Mycobacteria, or MRSA.
The Medical Research Council dyspnoea scale.
Spirometry.
Oxygen saturation levels.
Compliance with sputum clearance exercises.
How should you manage a person with an infective exacerbation of bronchiectasis?
Arrange hopsital admission if deemed needing IV antibiotics
Consider other diagnoses
Consider previous antibiotic use which may ahve led to resistant bacteria
Send sputum for culture and sensitivity testing before starting antibiotics but don’t delay treatment waiting for results
7-14 days of antibiotics
Ensure there’s a suitable airway clearance technique taught by a respiratory physiotherapist
Review response to empirical treatment when sputum culture and sensitivity results are available - if not clinical improvement, modify antibiotics
If significant breathlessness you can offer a trial of LABA
Consider prescribing a course of antibiotics for people to keep at home to facilitate prompt treatment of future exacerbations
What antibiotic treatment is suitable for an acute exacerbation?
Previous microbiology cultures should guide choice
Amoxicillin is first line if not available
Consider prescribing a course of antibiotics for people to keep at home to facilitate prompt treatment of future exacerbations
Do not routinely offer antibiotic prophylaxis to prevent acute exacerbations of bronchiectasis
If pt has 3 or more exacerbations per year they may be offered antibiotic prophylaxis e.g azithromycin (inhaled colistin if concurrent pseudomonas aeruginosa infection)
How does cystic fibrosis cause bronchiectasis?
Mutation in CFTR gene -> codes for Cl- channels which help facilitate Cl- out into the airway lumen -> water is reabsorbed together with Na+ -> thick mucous -> impaired mucociliary apparatus so impaired mucus clearance -> increases risk of infections and persistent inflammation
How does airway obstruction cause bronchiectasis? Explain in detail for a doctor
Airway obstruction -> endothelial damage -> mucociliary apparatus dysfunction -> increased risk of infection and inflammation -> chronic inflammation -> remodelling of bronchi
What is primary ciliary dyskinesia?
a rare, autosomal recessive genetic ciliopathy, that causes defects in the action of cilia lining the upper and lower respiratory tract, sinuses, Eustachian tube, middle ear, Fallopian tube, and flagella of sperm cells.
Within the first few months of life, most develop a chronic mucus-producing cough and runny nose.[3] The main consequence of impaired ciliary function is reduced or absent mucus clearance from the lungs, and susceptibility to chronic recurrent respiratory infections
What is Kartagener’s syndrome?
A triad:
Situs inversus (transposition of the viscera)
Abnormal frontal sinuses (producing sinusitis and bronchiectasis)
Primary ciliary dyskinesia
Why can hypogammaglobulinaemia cause bronchiectasis?
Defieicny of immunoglobulins -> increased susceptibility to infections of respiratory tract -> chronic inflammation and tissue damage
What is allergic bronchopulmonary aspergillosis? How can it cause bronchiectasis?
A hypersensitivity reaction to Aspergillus which typically affects pt with asthma or CF
It causes inflammation and damage to the airways which can leads to bronchiectasis
What will be found upon sputum sampling in bronchiectasis?
Neutrophilia (although in 25% of cases it may be eosinophilia)
Presence of bacteria
Increased mucus production
What is chronic colonisation?
the persistent presence of bacteria in the airways of individuals with bronchiectasis, even in the absence of acute infections. In bronchiectasis, the airways are often damaged and widened, which can result in the accumulation of mucus and secretions that can harbor bacteria. Chronic colonization can contribute to ongoing airway inflammation and exacerbations, and may also increase the risk of developing antibiotic resistance.
What are the most common bacteria associated with chronic colonisation in bronchiectasis?
Pseudomonas aeruginosa, Haemophilus influenzae, and Streptococcus pneumonia, Aspergillus, non-TB mycobacterium
What are the 3 most common causes of bronchiectasis in the UK?
Having had a lung infection in the past that damaged bronchi
Underlying problems with the immune system
Aspergillosis
What is tram track lines on CXR or CT?
Thickened bronchial walls
Whats gold standard for diagnosing bronchiectasis?
CT high resolution
Why are macrolide antibiotics best for bronchiectasis?
They have anti-inflammatory effects, immunomodulatory effects, broad-spectrum activity and low risk of resistance
When may surgical resection of a lung be indicated?
Localised disease that has failed to respond to medical therapy
Massive haemoptytsis
Lung cancer
Aspergillosis
How do you manage Allergic bronchopulmonary aspergillosis?
Oral glucocorticoids
What is allergic bronchopulmonary aspergillosis?
a fungal infection of the lung due to a hypersensitivity reaction to antigens of Aspergillus fumigatus after colonization into the airways
It predominantly affects pt with bronchial asthma and CF
What are the features of Allergic bronchopulmonary aspergillosis?
Bronchoconstriction - previous asthma or new wheeze/cough/dyspnoea
Recurrent chest infections
Bronchiectasis
What investigations should you do for Allergic bronchopulmonary aspergillosis?
eosinophilia
CXR
positive radioallergosorbent test to Aspergillus
positive IgG precipitins
raised IgE
How do you manage allergic bronchopulmonary aspergillosis?
Oral glucocorticoids
