Case 6 - Bronchiectasis, ABPA, CF and Massive Haemoptysis Flashcards
(54 cards)
1
Q
What is bronchiectasis?
A
- Chronic dilation of one or more bronchi
- Poor mucus clearance
- Therefore predisposition to recurrent/chronic bacterial infections
2
Q
Symptoms of bronchiectasis
A
- Chronic cough +/-haemoptysis, if massive bronchial artery could have been eroded - emergency
- Lots of phlegm - increases on postural change
- Progressive breathlessless
- Fever
- Night sweats
- Anorexia
- Weight loss
- Chest tighness
- Wheezing/pain
3
Q
Gold standard test for diagnosing bronchiectasis
A
High resolution CT scan - signet ring sign, dilated bronchi compared with artery
4
Q
How is lots of phlegm treated in bronchiectasis?
A
Postural drainage
5
Q
Typical organisms that cause post-infection bronchiectasis
A
- Staphylococcus aureus
- Haemophilus influenzae
- Bordetella pertussis - whooping cough
- Mycobacterium tuberculosis
- Viral - measles (as it causes post infectious pneumonia)
6
Q
Causes of bronchiectasisis
A
- **Post infection - eg TB, pneumonia, whooping cough
- Immunodeficiency - hypogammaglobulinaemia
- Genetic/mucociliary clearance defects**
- Obstruction - foreign body (eg dentures), tumour
- Toxic insult - gastric aspiration (esp after lung transplant), toxic gases
- Allergic bronchopulmonary aspergillosis
- Secondary immunodeficiency - HIV, cancer
- RA
- Associations - IBD, yellow nail syndrome
7
Q
Examples of genetic/mucociliary clearance defects causing bronchiectasis
A
- Cystic fibrosis
- Primary ciliary dyskinesia
- Young’s syndrome (triad of bronchiectasis, sinusitis and reduced fertility)
- Kartagener syndrome (triad of bronchiectasis, sinusitis and situs inversus)
8
Q
Examples of neoplasma which can cause obstructive bronchiectasis
A
- Bronchial carcinoid
- Bronchogenic carcinoma
9
Q
Examples of congenital conditions associated with bronchiectasis
A
- Mounier Kuhn syndrome
- William Campbell syndrome
- Bronchial atresia
- Pulmonary sequestration
- Alpha 1 anti-trypsin deficiency
10
Q
Blood tests to try and identify cause of bronchiectasis
A
- Immunoglobulin levels
- CF genotyping
- Aspergillus IgE/IgG and total IgE
- HIV test
- Rheumatoid factor
- Auto-antibodies
- Alpha-1 antitrypsin level
- Sputum cultures/bronchoscopy
- Spirometry and LFTs
11
Q
Classic radiological findings bronchiectasis
A
- Bronchus visualised within 1cm of pleural surface (failure to taper off)
- Bronchioarteriolar ratio >1.1 - Signet ring sign
- Tram track sign
12
Q
Common organisms present in bronchiectasis
A
- Haemophilus influenzae
- Psuedomonas aeruginosa
- Moraxella catarrhalis
- Stenotrophmonas maltophilia
- Fungi - aspergillus, candida
- Non-tuberculous mycobacterium
- Less common - staphylococcus aureus (CF more common)
13
Q
Signs of bronchiectasis
A
- Coarse crackles
- Wheeze
- Clubbing, cyanosis
- Muscle wasting
- Cor pulmonale signs - increased JVP, ascites, pedal oedema, pleural effusion, loud P2
14
Q
Radiological appearance of bronchiectasis (4 types)
A
- Cylindrical - failure of airway to taper off at distal edge
- Varicose
- Cystic
- Saccular - most severe, can have air fluid levels within sac
15
Q
Bronchiectasis management - general
A
- Treat underlying cause
- Physiotherapy - mucus and airway clearance
- Sputum routine culture + non tuberculour mycobacterium
- 10-14 days antibiotics according to cultures for acute exacerbations
- IV abx for severe infections
- Long term abx - prophylaxis
- Covid/flu vaccines
- Pulmonary rehabilitation if MRC dyspnoea score 3 or more
16
Q
Medical management for bronchiectasis
A
- Mucolytics eg carbocysteine, DNAse nebulisers
- Long term antibiotic - macrolides eg clarithromycin or nebulised abx eg colomycin
17
Q
Complications of bronchiectasis
A
- Frequent exacerbations and infections
- Poor lifestyle - limited by breathlessness, cough, haemoptysis, weight loss
- Metastatic abscess - bacteria seeds to brain
- Cor pulmonale
- Amyloidosis
18
Q
Common first line oral abx for bronchiectasis exacerbations
A
- Haemophilus influenzae - Amoxicillin (doxycycline if penicillin allergy)
- Pseudomonas aeruginosa - Ciprofloxacin (should counsel pts about rare side effect of achilles tendonitis)
19
Q
Criteria to identify bronchiectasis exacerbation vs normal baseline
A
A person with a deterioration in 3 or more key symptoms for at least 48hrs:
* Cough
* Sputum volume +/- consistency
* Sputum purulence
* Breathlessness +/- exercise tolerance
* Fatigue
* Haemoptysis
20
Q
What is allergic bronchopulmonary aspergillosis?
A
Type 1 and 3 hypersensitivity reaction caused by inhalation of aspergillus fumigatus spores - commun fungus indoors and outdoors
NOT a fungal infection
21
Q
What occurs from ABPA?
A
Repeated damage to lung (often upper lobe) from immunological reactions
22
Q
Who is ABPA often seen in?
A
- Asthma
- Bronchiectasis
- Cystic fibrosis
23
Q
Diagnosis of ABPA
A
- Symptoms - dry cough and wheeze
- Positive bloods
24
Q
Positive bloods for ABPA
A
- Raised aspergillus IgE
- High total IgE
- High eosinophil level too
25
Treatment for ABPA
Steroids may be recquired if ongoing symptoms and high total IgE level
26
Define CF
* Autosomal recessive disease
* Mutation in CFTR gene
* = multisystem disease
* Commonly affecting respiratory and GI system
* Thickened bodily secretions
27
CF pathophysiology
* Long arm Chromosome 7 defect
* CFTR mutation
* = Ineffective cell surface Cl- transport
* Thick dehydrated bodily fluids
28
Most common CF mutation
DeltaF508
29
Reasons for better prognosis of CF patients now
* Improved nutrient replacement
* Newborn screening
* Prophylactic abx
* Realisation of other micro-organisms colonisation
* Newer therapies - potentiators, modulators
30
What are the 6 classes of CF mutation?
1. Protein synthesis defect
2. Maturation defect
3. Gating defect
4. Conductance defect
5. Reduced quantitiy
6. Reduced stability
31
CF diagnostic criteria
One or more of phenotypic features:
* History of CF in sibling
* Positive newborn screening test result
AND one of following:
* An increased sweat chloride concentration (>60mmol/L) on SWEAT TEST
* Identification of two CF mutations - genotyping
* Demonstration of abnormal nasal epithelia ion transport (nasal potential difference)
32
Conditions which CF can present with
* Meconium ileus
* Intestinal malabsorption
* Recurrent chest infections
* Newborn screening
33
What is meconium ileus?
In 15-20% of babies born with CF the bowel is blocked by sticky secretions
Signs of intestinal obstruction occur soon after birth such as:
* Bilious vomitting
* Abdo distension
* Delay in passing meconium
34
How does intestinal malabsorption occur in CF?
* 90% of individuals with CF have this
* Evident in infancy
* Main cause is severe deficiency in pancreatic enzymes
35
Systemic effects of CF
* Head - nasal polyps, recurrent sinusitis
* Chest - recurrent chest infections, bronchiectasis, abnormal sweat secretions (high NaCl)
* GI - liver disease, portal HTN, gall stones, pancreatic insufficiency, diabetes, distal intestinal obstruction syndrome, steatorrhoea
* Male infertility
* Osteoporosis
* Finger clubbing
36
Common CF complications
* Respiratory infections
* Low body weight
* Distal intestinal obstruction syndrome (DIOS)
* CF related diabetes mellitus
37
Respiratory infection complication from CF
* Need aggressive therapy with physio and abx
* Patients often receive prophylactic abx to maintain health
38
Low body weight complication from CF
* Need to carefully monitor
* Consequence of pancreatic insufficiency (no enzymes) therefore pts may need pancreatic enzyme replacement therapy
* May need NG or PEG feeding
39
DIOS complication from CF
* DIOS vs constipation - faecal obstruction in ileocaecum versus whole bowel
* Distal ileum = thick, dehydrated faeces
* Often presents with palpable RIF mass (faeces)
40
Diagnosis of DIOS
* Palpable RIF mass
* AXR demonstrating faecal loading at junction of small and large bowel
41
Treatment for DIOS
PO Gastrografin - draws water across the bowel wall via osmosis into the bowel lumen = rehydrate the dry faecal mass --> allows to pass
42
CF lifestyle advice
* Stop smoking
* Avoid other CF patients - spread infections
* Avoid people with colds/infections
* Avoid jacuzzis - pseudomonas
* Clean and dry nebs regularly
* Avoid stables, compost or rotting vegetation - risk of aspergillus fumigatus inhalation
* Annual flu vaccine
* NaCl tablets in hot weather/vigorous exercise
43
CF management
* As per bronchiectasis guidelines - physio ofr airway clearance
* Exercise
* Mucolytic treatment - inc nebulised DNase (pulmoenzyme)
* Pancreatic enzyme replacement therapy (eg Creon)
* Nutritional supplement if underweight
* Fat soluble vitamins ADEK
* Long term abx - inhaled or nebs
* Optimisation of CF related diabetes
* Long term monitoring for CF related diabetes, liver disease and osteoporosis
44
Newer therapies for CF
* Novel CFTR modulators/potentiatios (eg Kaftrio)
* These have shown excellent efficacy with improvements of FEV1, weight, quality of life and reduction of freq of infective exacerbations
45
Surgical treatment for CF if possible
Lung transplant
But if colonised with pseudomonas or Burkholderia cepacia this affects transplant eligibility
46
Define massive haemoptysis
* Is >240mls in 24hrs (100-600mls in 24hrs)
OR
* Is >100mls / day over consecutive days
47
Management of massive haemoptysis
* A-E approach - EMERGENCY
* Lie patient on side of suspected lesion (if known via CT or side of pain)
* Stop NSAIDs / aspirin / anticoags
* Oral tranexamic acid fo 5 days or IV
* Consider Vit K
* Abx if evidence of infection
* CT aortagram - interventional radiologist may be able to do bronchial artery embolisation`
48
Common cause of massive haemoptysis from infection
* Irriated bronchial artery due to infection
49
How does massive haemoptysis cause death?
Asphysxia rather than blood loss
50
Symptoms of massive haemoptysis
* Extreme aniety
* SOB
* Hypoxia +/- cyanosis
* Vomitting - if blood swallowed
51
Common causes of massive haemoptysis
* Infection
* Bronchiectasis
* TB
* Lung abscess
* Aspergilloma (fungal ball)
* Malignancy
52
Rare cause of massive haemoptysis
* PE
* Systemic vasculitis
* AV malformation
* AV fistulae
* Cardiac cause / mitral stenosis
* Thrombolytic therapy
53
Why do we lie pt on side of suspected lesion in massive haemoptysis?
* Gravity causes blood to pool on that side
* Stays in that lung and does not track up into other lung
* Maintains V/Q in healthy lung
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