Bronchiectasis Flashcards
What is it?
Irreversible and abnormal chronic dilatation of one or more bronchi causing poor mucus clearance
there is a predisposition to recurrent or chronic bacterial infection
What are the main causes of bronchiectasis?
- Immunodeficiency
- Post infective
- Mucociliary clearance genetic defects
- Bronchial obstruction- - lung tumour, foreign body, extrinsic lymph node
- Toxic insult- gastric aspiration, toxic chemicals /gases inhaled
- Allergic bronchopulmonary aspergillosis (ABPA)
- RA
What infections predispose to bronchiectasis?
whooping cough, TB, pneumonia
Eg. typical story is recurrent childhood pulmonary infections
Which immunodeficiencies predispose to bronchiectasis?
-
Primary:
- Panhypogammaglobulinaemia
- IgA deficiency
- IgG deficiency
-
Secondary:
- HIV
- Malignancy
Which Mucociliary clearance genetic defects
predispose to bronchiectasis?
- cystic fibrosis
- primary ciliary dyskinesia (PCD)
- Young’s syndrome
- Kartagener syndrome
What is youngs syndrome?
bronchiectasis, sinusitis, reduced fertility (associated with mercury exposure)
What is Kartagener syndrome?
Kartagener syndrome- bronchiectasis, sinusitis and situs inversus (PCD + situs inversus)
What does bronchiectasis have associations with
IBD, Yellow nail syndrome
What are common organisms causing Bronchiectasis exacerbation?
Haemophillus influenzae
Pseudonomas aeruginosa
Moraxella Catarrhalis
Stenotrophomonas maltophilia
Fungi- Aspergillus, Candida
Non- tuberculosis mycobacteria
Less common- Staph aureus (CF)
How to diagnose a acute exacerbation of disease?
A person with bronchiectasis with a deterioration in 3 or more key symptoms for at least 48 hours:
- Cough
- Sputum volume/purulence
- Sputum colour/ consistancy
- SOB and exercise tolerance
- fatigue
- haemoptysis
Differential diagnosis?
asthma, COPD and malignancies
Symptoms in brnchiectasis
- Chronic cough
- Persistent large sputum production (Eggcups)
- SOB
- Haemoptysis
- Weight loss/ fatigue/ exercise limitation
Signs of examination suggesting bronchiectasis
- Crackles
- High pitched inspiratory squeaks
- Wheeze
- Clubbing(rare)
How to diagnose bronchiectasis? What results will you get?
High-resolution CT (GOLD STANDARD)- Tram-tracks and signet ring signs
CXR- Tram-track airways (bronchial wall thickening) and ring shadows. Can be normal CXR
Blood tests to find cause of newly diagnosed bronchiectasis?
- Immunoglobulin levels- immunodeficiency causes
- Cystic fibrosis genotype
- Aspergillus specific IgE/IgG and serum total IgE
- HIV test
- Rheumatoid factor- RA
- Autoantibodies - (might be rheumatic related)- ANA, anti-CCP, RF
- Alpha-1-antitrypsin level
FBC
U&E
What other investigations can be done?
Sputum sample- M, C and S and for nontuberculous mycobacteria- for all patients and during acute exacerbation
Pt with localised disease on imaging - bronchoscopy - check from bronchial obstruction
how to check for primary ciliary dyskinesia
features of primary ciliary dyskinesia:
early-onset of symptoms (in childhood/ teenage years), otitis media, rhinosinusitis and male infertility
Management overview?
Educate the patient on their condition
Help with smoking cessation, diet and exercise
Immunisations: yearly flu vaccine
Treat underlying cause
Eg. Bronchodilators in patients with coexisting asthma/COPD or in those with significant breathlessness.
Reduce the number of exacerbations
Physiotherapy- mucus/airway clearance
Mucoactives/lytics
Treat acute exacerbations with 10-14 day antibiotics
Main organisms causing acute exacerbation and antibiotic treatment?
and for how long?
Side effects if any?
Antibiotics for 10-14 days - use sputum cultures/sensitivities for acute exacerbations
- Haemophilus influenzae- Oral Amoxicillin/Doxy (Ax)
- Pseudomonas Aerug- Oral Ciprofloxacin- may get achilles tendonitis
In severe acute exacerbation infections give
IV antibiotics
Long-term (prophylactic) antibiotics used in :
for pts with recurrent infective exacerbations(> 3 per year)
despite education, treatment of underlying causes, physiotherapy (+/- mucoactives) should be considered for long term antibiotics.
Pulmonary rehabilitation is advised if :
MRC dyspnoea score >= 3
Complications that can occur
- Recurrent infections
- Infective exacerbations
- Chronic respiratory failure
- Haemoptysis(may be massive and life-threatening)
- Cor pulmonale
- Pneumothorax
- Chest pain
Allergic Bronchopulmonary Aspergillosis (ABPA) is caused by:
Caused by aspergillus fumigatus exposure
Aspergillus is a common fungus found indoors and outdoors
ABPA Pathophysiology
ABPA is a combination of types 1 and 3
hypersensitivity reactions following inhalation of
fungal spores i.e. it is not a fungal infection
Repeated damage from these immunological
reactions leads to bronchiectasis (often upper lobe)
Who is ABPA seen in?
ABPA is seen more in patients with Asthma,
Bronchiectasis and Cystic Fibrosis
How is ABPA diagnosed ?
Combination of symptoms (often dry cough and wheeze)
Positive blood tests (raised Aspergillus IgE level as well as a high Total IgE – these are often accompanied by a high eosinophil level too)
Treatment of ABPA?
Steroids may be required if ongoing
symptoms and high Total IgE level
