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Respiratory YR3 > Case 6 - Bronchiectasis, ABPA, CF and Massive Haemoptysis > Flashcards

Case 6 - Bronchiectasis, ABPA, CF and Massive Haemoptysis Flashcards

1
Q

What is bronchiectasis?

A
  • Chronic dilation of one or more bronchi
  • Poor mucus clearance
  • Therefore predisposition to recurrent/chronic bacterial infections
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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
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3
Q

Gold standard test for diagnosing bronchiectasis

A

High resolution CT scan - signet ring sign, dilated bronchi compared with artery

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4
Q

How is lots of phlegm treated in bronchiectasis?

A

Postural drainage

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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)
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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
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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)
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8
Q

Examples of neoplasma which can cause obstructive bronchiectasis

A
  • Bronchial carcinoid
  • Bronchogenic carcinoma
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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
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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
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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
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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)
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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
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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
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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
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16
Q

Medical management for bronchiectasis

A
  • Mucolytics eg carbocysteine, DNAse nebulisers
  • Long term antibiotic - macrolides eg clarithromycin or nebulised abx eg colomycin
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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
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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)
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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

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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

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21
Q

What occurs from ABPA?

A

Repeated damage to lung (often upper lobe) from immunological reactions

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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
Q

Treatment for ABPA

A

Steroids may be recquired if ongoing symptoms and high total IgE level

26
Q

Define CF

A
  • Autosomal recessive disease
  • Mutation in CFTR gene
  • = multisystem disease
  • Commonly affecting respiratory and GI system
  • Thickened bodily secretions
27
Q

CF pathophysiology

A
  • Long arm Chromosome 7 defect
  • CFTR mutation
  • = Ineffective cell surface Cl- transport
  • Thick dehydrated bodily fluids
28
Q

Most common CF mutation

A

DeltaF508

29
Q

Reasons for better prognosis of CF patients now

A
  • Improved nutrient replacement
  • Newborn screening
  • Prophylactic abx
  • Realisation of other micro-organisms colonisation
  • Newer therapies - potentiators, modulators
30
Q

What are the 6 classes of CF mutation?

A
  1. Protein synthesis defect
  2. Maturation defect
  3. Gating defect
  4. Conductance defect
  5. Reduced quantitiy
  6. Reduced stability
31
Q

CF diagnostic criteria

A

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
Q

Conditions which CF can present with

A
  • Meconium ileus
  • Intestinal malabsorption
  • Recurrent chest infections
  • Newborn screening
33
Q

What is meconium ileus?

A

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
Q

How does intestinal malabsorption occur in CF?

A
  • 90% of individuals with CF have this
  • Evident in infancy
  • Main cause is severe deficiency in pancreatic enzymes
35
Q

Systemic effects of CF

A
  • 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
Q

Common CF complications

A
  • Respiratory infections
  • Low body weight
  • Distal intestinal obstruction syndrome (DIOS)
  • CF related diabetes mellitus
37
Q

Respiratory infection complication from CF

A
  • Need aggressive therapy with physio and abx
  • Patients often receive prophylactic abx to maintain health
38
Q

Low body weight complication from CF

A
  • 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
Q

DIOS complication from CF

A
  • DIOS vs constipation - faecal obstruction in ileocaecum versus whole bowel
  • Distal ileum = thick, dehydrated faeces
  • Often presents with palpable RIF mass (faeces)
40
Q

Diagnosis of DIOS

A
  • Palpable RIF mass
  • AXR demonstrating faecal loading at junction of small and large bowel
41
Q

Treatment for DIOS

A

PO Gastrografin - draws water across the bowel wall via osmosis into the bowel lumen = rehydrate the dry faecal mass –> allows to pass

42
Q

CF lifestyle advice

A
  • 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
Q

CF management

A
  • 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
Q

Newer therapies for CF

A
  • 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
Q

Surgical treatment for CF if possible

A

Lung transplant
But if colonised with pseudomonas or Burkholderia cepacia this affects transplant eligibility

46
Q

Define massive haemoptysis

A
  • Is >240mls in 24hrs (100-600mls in 24hrs)
    OR
  • Is >100mls / day over consecutive days
47
Q

Management of massive haemoptysis

A
  • 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
Q

Common cause of massive haemoptysis from infection

A
  • Irriated bronchial artery due to infection
49
Q

How does massive haemoptysis cause death?

A

Asphysxia rather than blood loss

50
Q

Symptoms of massive haemoptysis

A
  • Extreme aniety
  • SOB
  • Hypoxia +/- cyanosis
  • Vomitting - if blood swallowed
51
Q

Common causes of massive haemoptysis

A
  • Infection
  • Bronchiectasis
  • TB
  • Lung abscess
  • Aspergilloma (fungal ball)
  • Malignancy
52
Q

Rare cause of massive haemoptysis

A
  • PE
  • Systemic vasculitis
  • AV malformation
  • AV fistulae
  • Cardiac cause / mitral stenosis
  • Thrombolytic therapy
53
Q

Why do we lie pt on side of suspected lesion in massive haemoptysis?

A
  • 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
54
Q
A

Respiratory YR3 (14 decks)

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