bronchiectasis Flashcards
define
= chronic infection of bronchi + bronchioles leading to:
- permanent [chronic] bronchial dilation
- impaired mucocilliary clearance
- freq bacterial infections
pathophys
= chronic infection of bronchi/bronchioles=> permanent dilation
retained inflam secretions + microbes => airway damage and recurrent infections
organisms:
- h. influenza
- pneumococcus
- s.aureus
- pseudemonas
causes: main groups
congenital
post infectious
immunodeficiency
other
congenital causes
cf [mainly upper lobe filtration]
kartagener’s/ PCD
young’s syndrome [azoospermia + bronchiectasis]
post infectious
measles,
pertussis
pneumonia
TB
bronchiolitis
immunodeficiiency
hypogammaglobinemia
- x-linked a-gamablobinemia: Bruton’s [leads to severe block in B cell development]
- CVID [common variable immunodeficiency= get low Ab levels in blood]
- IgG subclass deficiency
- IgaA deficiency
Other:
- bronchial obstruction= LNs, tumour, FB
- ABPA
- RA
- UC
- Yellow nail syndrome
- yellow nail discolouration + atrophy
- lymphoedema
- pleural effusions
- bronchiectasis
s/s
symptoms:
- persistent cough w/ purulent sputum
- intermittent haemoptysis [may be massive]
- fever, wt loss
signs:
- clubbing
- course inspiratory crackles - usually @ bases, shifts w/ coughing
- wheeze [asthma, copd, abpa]
- purulent sputum
- of a cause:
- situs inversus [+PCD = kartagener’s]
- splenomegaly cos of immunodeficiency
complications
2 new [pneu] pul[led] apple[s], crap massively eeeee
2 new = pneumonia, pneumothorax
pul[lled] = pulmonary htn
apple** = pleural effusion
CRAP - cerebral abscess, r, amyloidosis, persistent infections
massively- massive hemoptysis
eeee- empyema
1. full blood count - blood eosinophilia is common in allergic bronchopulmonary aspergillosis
2.sputum examination:
- obtained by deep coughing, physiotherapy or aerosol inhalation
- with the naked-eye to confirm patients account
- bacteriological examination
- tests for mycobacteria are essential in all children and adults
3. chest radiography:
- to exclude obvious localised lung disease
- note that only about 50% of patients with bronchiectasis does a chest X-ray show the characteristic features of thickened bronchial walls (seen as parallel ‘tramlines’, signet ring or curvilinear opacities), cystic lesions with fluid levels, areas of collapse with crowding of pulmonary vasculature, or scarring
3. lung function tests to assess extent of lung damage
- in children (usually >5 years) - FEV1, FVC, forced expiratory flow (FEF)
- in adults - FEV1, FVC, peak expiratory flow PEF
- serum immunoglobulin determination - should be done at presentation by measuring serum IgG, IgA and IgM levels and serum electrophoresis
5. screening for cystic fibrosis
- done in all children and adults up to 40 years of age and adults over 40 years with clinical features suggestive of cystic fibrosis
- two measurements of sweat chloride
- CFTR genetic mutation analysis
6. skin prick test for aspergillus
7. high resolution CT scan: ******************
- is the investigation of choice to confirm the diagnosis
- used in patients in whom bronchiectasis is suspected but the X-ray is unhelpful
- characteristic fetures seen in HRCT are
- bronchial wall dilation (internal diameter of the lumen is bigger than the diameter of the accompanying bronchial artery)
- lack of bronchial tapering
8. bronchoscopy is indicated to investigate haemoptysis or to exclude an obstructing lesion in localised bronchiectasis
9. gastrointestinal investigations such as 24-hour pH monitoring, barium studies
- the threshold for gastrointestinal investigations should be low for children due to the higher incidence of structural abnormalities
- gastric aspiration should also be considered as a cause after lung transplantation
10. urinanalysis:
amyloidosis may cause proteinuria
mx
Bronchiectasis describes a permanent dilatation of the airways secondary to chronic infection or inflammation. After assessing for treatable causes (e.g. immune deficiency) management is as follows:
- physical training (e.g. inspiratory muscle training) - has a good evidence base for patients with non-cystic fibrosis bronchiectasis
- postural drainage
- antibiotics for exacerbations + long-term rotating antibiotics in severe cases
- bronchodilators in selected cases
- immunisations
- surgery in selected cases eg. if severe hemoptysis: bronchial artery embolisation (e.g. Localised disease= localised resection; lung transplant/heart-lung translplant)
Most common organisms isolated from patients with bronchiectasis:
- Haemophilus influenzae (most common)
- Pseudomonas aeruginosa
- Klebsiella spp.
- Streptococcus pneumoniae
