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Y5 Respiratory System > List II - Less Common 'Know of' Conditions > Flashcards

List II - Less Common 'Know of' Conditions Flashcards

1
Q

What is bronchiectasis?

A
  • Widening of bronchi or their branches
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2
Q

What are the causes of bronchiectasis?

A
  • Congenital
  • Cystic fibrosis
  • Young’s sydrome
  • Primary cilliary dyskinesia
  • Kartagener’s syndrome - dextrocardia
  • Post infection
  • Measles
  • Bordetella pertussis (whooping cough as a child)
  • Bronchiolitis
  • Pneumonia
  • TB
  • HIV
  • Other
  • Bronchial obstruction - tumour or foreign body
  • ABPA?
  • Hypogammaglobulinaemia
  • RA
  • UC
  • Idiopathic
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3
Q

What is the pathophysiology of bronchiectasis?

A
  • Chronic infection of bronchi and bronchioles
  • Haemophilus influenzae
  • Streptococcus pneumoniae
  • Staphylococcus aureus
  • Pseudomonas aeruginosa
  • Permanent dilation of these airways
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4
Q

What are the presenting features of bronchiectasis?

A

Symptoms

  • Persistent cough
  • Copious purulent sputum
  • Intermittent haemoptysis

Signs

  • Clubbing
  • Coarse inspiratory crackles
  • Wheeze - asthma, COPD, ABPA
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5
Q

What are the appropriate bloods for bronchiectasis?

A
  • Serum Ig’s

* Aspergillus serology

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6
Q

What are the appropriate radiological investigations of bronchiectasis?

A
  • Chest x-ray
  • Cystic shadows
  • Thickened bronchial walls (tramline, ring shadows)
  • High resolution CT
  • Assess extent/distribution
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7
Q

What other investigations can be done for bronchiectasis?

A
  • Sputum MC and S
  • Lung function - often show obstructive pattern (assess reversibility)
  • Bronchoscopy - to locate site of haemoptysis or exclude obstruction
  • CF sweat test if suspected
  • Aspergillus skin prick test
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8
Q

What is the conservative management of bronchiectasis?

A
  • Chest physiotherapy - aid sputum expectoration and mucous drainage
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9
Q

What is the medical management of bronchiectasis?

A
  • Postural drainage x 2 per day
  • Antibiotics - according to bacterial sensitivities (if pseudomonas is cultures require either ciprofloxacin po or IV abx)
  • Bronchodilators - nebulised salbutamol (if asthma, COPD, CF, ABPA)
  • Corticosteroids - prednisolone for ABPA
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10
Q

What is the surgical management of bronchiectasis?

A
  • Indications

- May be indicated in localised disease or to control severe haemptysis

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11
Q

What are the possible complications of bronchiectasis?

A
  • Pneumonia
  • Pleural effusion
  • Pneumothorax
  • Haemoptysis
  • Cerebral abscess
  • Amyloidosis
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12
Q

What is the mechanism of carbon monoxide poisoning?

A
  • Carbon monoxide has a high affinity for haemoglobin and myoglobin
  • Results in a left shifting of the oxygen dissociation, early plateau in the curve and tissue hypoxia
  • Approximately 50 deaths per year in the UK from accidental carbon monoxide poisoning
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13
Q

Which population groups are at risk of carbon monoxide poisoning?

A
  • Badly maintained houses e.g. student housing
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14
Q

What are the clinical features of carbon monoxide poisoning?

A
  • Heachache 90% of cases
  • Nausea and vomiting 50%
  • Vertigo 50%
  • Confusion 30%
  • Subjective weakness 20%
  • Severe toxicity - pink skin and mucosae, hyperpyrexia, arrhythmias, extrapyramidal features, coma and death
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15
Q

What are the investigations required for carbon monoxide poisoning?

A
  • Pulse oximetry may be falsely high due to similarities between oxyhaemoglobin and carboxyhaemoglobin
  • Therefore a VBG or ABG should be taken
  • Typical carboxyhaemoglobin levels:
  • <3% non-smokers
  • <10% smokers
  • 10-30% symptomatic - headache, vomiting
  • > 30% severe toxicity
  • ECG is useful supplementary investigation to look for cardiac ischaemia
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16
Q

What is the management for patients with carbon monoxide poisoning?

A
  • All patients with suspected carbon monoxide poisoning should be assessed in the emergency department
  • 100% high flow oxygen via a NRBM
  • This decreases the half life of carboxyhaemoglobin (COHb)
  • Should be administered as soon as possible, with treatment continuing for a minimum of 6 hours
  • Target O2 sats are 100%
  • Treatment is generally continued until all symptoms have resolved, rather than monitoring CO levels
  • Hyperbaric oxygen
  • Due to the small number of cases the evidence base is limited, but there is some evidence that long term outcomes may be better than standard oxygen therapy for more severe cases
  • Discussion with a specialist should be considered for more severe cases (e.g. levels >25%)
  • 2008 Department of Health ‘Recognising Carbon Monoxide Poisoning’ listed LOC at any point, neurological signs other than headache, myocardial ischaemia or arrhythmia and pregnancy as indications for hyperbaric oxygen
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17
Q

What is idiopathic pulmonary fibrosis?

A
  • Type of idiopathi interstitial lung disease - inflammation and fibrosis of distal airspaces (defined histologically)
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18
Q

What is the aetiology of IPF?

A
  • Unknown
  • Associations with:
  • Asbestos
  • Hard metals
  • Drugs
  • Radiation
  • AI disorders (systemic sclerosis, RA, SLE, Sjorgrens, UC, chronic active hepatitis, polymyositis, dermatomyositis)
  • Genetic
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19
Q

How common is IPF?

A
  • More common in older age
  • M>F
  • 20/100,000
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20
Q

What are the histological features of IPF?

A
  • Organising inflammatory cell infiltrate
  • Fibroblast proliferation
  • Progressive alveolar wall thickening
  • Reduced lung volumes
  • Impaired diffusion
  • Respiratory failure eventually develops
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21
Q

What are the types of IPF?

A
  • Usual interstitial pneumonia
  • Inflammatory cells in air spaces - fibrosis leads to lung tissue contraction - honey combing
  • Desquamative interstitial pneumoniae
  • Predominant infiltrate is mononuclear cell, fibrosis less prominent
  • Hamman-Rich syndrome
  • Predominantly inflammatory alveolitis of acute onset (better response to therapy)
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22
Q

What are the presenting clinical features of IPF?

A

Symptoms

  • Gradual onset of SOB (exertional commonly)
  • Non-productive cough
  • Malaise
  • Weight loss
  • Arthralgia
  • Asymptomatic 5%

Signs

  • Clubbing 50%
  • Respiratory distress
  • Fine end inspiratory crackles

Complications
* Cor pulmonale (if severe raised JVP, peripheral oedema, systolic parasternal heave, loud P2)

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23
Q

What are the differential diagnoses for IPF?

A

Pulmonary fibrosis:

  • Drugs
  • Amiodarone
  • Nitrofurantoin
  • Disease
  • Idiopathic
  • AS
  • Sarcoidosis,
  • Histoplasmosis
  • TB
  • Asbestosis
  • Silicosis (foundry workers)
  • Extrinsic allergic
  • Alveolitis (hypersensitivity pneumonitis)
  • Iatrogenic
  • Radiation fibrosis
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24
Q

What are the appropriate blood investigations for IPF?

A
  • CRP
  • Ig’s
  • ANA 30% +ve
  • RhF 10% +ve
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25
Q

What are the appropriate radiological investigations for IPF?

A
  • Chest x-ray
  • Similar to intersitital oedema of LVF but w/o cardiomegaly): diffuse/mainly basal and peripheral ground-glass change, bilateral lower-zone reticulonodular shadows, honeycombing (advanced)
  • High resolution CT
  • Similar to x-ray changes but more sensitive
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26
Q

What further investigations can be done for IPF?

A
  • Lung function - ↓FVC, FEV1/FVC normal/↑, impaired gas transfer (markers of disease progression: gas transfer, total lung capacity, alveolar volume)
  • Tissue biopsy - typical cellular infiltrate/fibrosis, patchy changes (so transbronchial biopsy may be inadequate)
  • ABG - may show low PaO2, high PaCO2
  • BAL - May indicate activity of alveolitis
  • Raised lymphocytes - good prognosis
  • Raised neutrophils - poor response/prognosis
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27
Q

What are the conservative management measures of IPF?

A
  • Supportive
  • O2 for SOB
  • Diuretics for fluid retention
  • Antibiotics for infection
  • Pulmonary rehabilitation
  • Opiates

+/- Palliative care

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28
Q

What is the medical management options for IPF?

A
  • Immunosuppressants
  • Azathioprine 2 mg/kg
  • Interferon?
  • Do not use high dose steroids (unless diagnosis is in doubt)
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29
Q

What are the surgical options for IPF?

A
  • Lung transplant - if young with advanced disease
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30
Q

What are the complications of IPF?

A
  • Risk of lung cancer 10% cases

* Respiratory failure

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31
Q

What is the prognosis of IPF?

A
  • Lung function may stay stable for many months
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32
Q

What is the mortality of IPF?

A
  • Median survival (from diagnosis) 3 years (despite intervention)
  • 5 year survival 50% (range 1-20 years)
  • 1500 deaths / year in UK
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33
Q

What is coal workers pneumoconiosis?

A
  • Interstitial lung disease
  • Common dust disease in countries that have/had underground coal mines
  • Results from inhalation of coal dust particles 1-3 cm in diameter over 15-20 year period
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34
Q

What are the risk factors for coal workers pneumoconiosis?

A
  • Coal mining occupation
  • Smoking
  • Urban dwellers
35
Q

How common is coal workers pneumoconiosis?

A
  • 2% in coal miners
36
Q

What is the pathophysiology of coal workers pneumoconiosis?

A
  • Coal dust particles ingested by macrophages
  • Macrophages die
  • Release their enzymes
  • Leads to fibrosis
37
Q

What are the types of coal workers pneumoconiosis?

A
  • Asymptomatic anthracosis
  • Accumulated carbon pigment without cellular reaction (common in urban dwellers, smokers, coal miners)
  • Simple coal workers pneumoconiosis (CWP)
  • Coal macules form within lung
  • Develops into nodules
  • Progressive massive fibrosis
  • Due to progression of CWP
  • Extensive fibrosis develops with pulmonary nodules that coalesce over the years
  • Central necrosis may occur leading to melanoptysis (black sputum)
  • Centrilobular empysema
  • Can occur in non-smokers
  • Caplan’s syndrome
  • RA plus any of the pneumoconioses and pulmonary rheumatoid nodules
38
Q

What are the clinical features of coal workers pneumoconiosis?

A

Symptoms -

  • Asymptomatic/co-existent chronic bronchitis (CWP)
  • Progressive SOB (PMF)
  • Melanoptysis (PMF)

PMH - RA

Signs -

  • RA
  • Cor pulmonale (advanced PMF)
  • Raised JVP
  • Cyanosis
  • Parasternal heave
  • Loud P2
39
Q

What are the appropriate blood investigations for coal workers pneumoconiosis?

A
  • RhF often +ve
40
Q

What are the appropriate radiological investigations for coal workers pneumoconiosis?

A
  • Chest x-ray
  • May round discrete nodules/opacities (1-10mm simple CWP especially in the upper zone)
  • Fibrotic masses/massive pulmonary fibrosis with homogenous shadowing (PMF)
  • Large opacities with necrosis/cavitation (PMF)
41
Q

What further investigations can be done for coal workers pneumoconiosis?

A
  • Lung function tests
  • Remains preserved unless PMF occurs → restrictive defect with ↓lung volume and ↓DLCO (but obstructive picture may also occur if centrilobular emphysema occurs)
42
Q

What is the management of coal workers pneumoconiosis?

A
  • Patient education
  • Stop smoking
  • Avoid exposure to coal dust
  • Symptomatic
  • Supportive
  • Therapy for any co-existing chronic bronchitis
  • Compensation
  • Often eligible to claim under the UK Industrial Injuries Act
43
Q

What are the risks of coal workers pneumoconiosis?PMF?

A
  • Can lead to respiratory failure and death
44
Q

What is the prognosis of coal workers pneumoconiosis?

A
  • Relatively good for simple CWP (poor if PMF)
45
Q

What is asbestosis?

A
  • Type of industrial lung disease

* Lung fibrosis causes by prolonged asbestos exposure (a mineral fibre of silicate origin)

46
Q

What is the aetiology of asbestosis?

A
  • Caused by inhalation of asbestos fibres
  • Risk factors include
  • Occupations such as ship building, construction, plumbing, welding
  • Occupation methods such as fire proofing, pipe lagging e.g. Hoffman presses in textile factories), electrical wire insulation and roofing felt
  • Age - born between 1945-1950
47
Q

How common is asbestosis?

A
  • Was widespread in Europe until 1980’s - peak incidence likely to be 2020 (owing to 40 year latent period)
48
Q

What are the types of asbestosis?

A
  • Chrysotile - white asbestos - least fibrogenic
  • Amosite - brown asbestos - least common, has intermediate fibrogenicity
  • Crocidolite - blue asbestos - most fibrogenic
49
Q

What is the pathophysiology of asbestosis?

A
  • Asbestos bodies - asbestos fibres coated with brown phagocyte ferritin consequent upon being engulfed by macrophages on histology pulmonary fibrosis related to degree of exposure
50
Q

What are the clinical symptoms of asbestosis?

A
  • Similar to other fibrotic lung disease
  • Progressive SOB
  • Productive cough
51
Q

What are the signs of asbestosis?

A
  • Finger clubbing

* Fine end expiratory crackles basal

52
Q

What the appropriate radiological investigations for asbestosis?

A
  • Chest x-ray
  • Initially linear opacities in lower zones - fibrotic changes in costophrenic and cardiophrenic angles (starting in lower lobes subpleurally and progressing proximally)
  • Calcified pleural plaques on diaphragmatic/lateral pleural surfaces (fibrosis occurring in absence of plaques is unlikely to be due to asbestos exposure
  • Pleural thickening/effusions
  • CT scan
  • Peripheral septal lines
  • Bronchiolar thickening
  • Honey combing
  • Pleural thickening
  • Pleural plaques (density of parenchymal abnormalities correlates well with severity of symptoms)
53
Q

What can be seen on lung functions tests in a patient with asbestosis?

A
  • Restrictive defect occurs with reduced gas exchange (DLCO)
54
Q

What is the approach to management of asbestosis?

A
  • Patient education
  • Stop smoking - can significantly reduce risk of cancer
  • Patients often eligible for the compensation under the UK Industrial Injuries Act
55
Q

What are the ongoing risks of asbestosis?

A
  • Pulmonary fibrosis
  • Benign pleural plaques
  • Pleural effusion
  • Bronchial adenocarcinoma - especially in smokers
  • Malignant mesothelioma
56
Q

What is the prognosis of asbestosis?

A
  • Variable - ranging from stable (mild symptoms) to progressive deterioration and respiratory failure (progression usually slows after a decade of non-exposure)
57
Q

What is the mortality of patients with asbestosis?

A
  • Lung cancer is the most common cause of death in patients with asbestosis
58
Q

What is malignant mesothelioma?

A
  • Type of industrial lung disease

* Tumour of mesothelial cells of the pleura (rarely in pericardial/peritoneal cavities or other organs)

59
Q

What is the cause of mesothelioma?

A
  • Inhalation of asbestos fibres - complex as 90% report having previous exposure but 20% have pulmonary asbestosis
60
Q

What are the risk factors for mesothelioma?

A
  • Risk factors include
  • Occupations such as ship building, construction, plumbing, welding
  • Occupation methods such as fire proofing, pipe lagging e.g. Hoffman presses in textile factories), electrical wire insulation and roofing felt
  • Age - born between 1945-1950
61
Q

How common is mesothelioma?

A
  • Uncommon
  • Incidence 8/100,000/year in the UK
  • M>F
62
Q

What is the pathophysiology of mesothelioma?

A
  • Unlike asbestosis, there is no threshold for the risk of mesothelioma
  • Histological subtypes
  • Epithelioid
  • Sarcomatoid
  • Biphasic (epithelioid and sarcomatoid)
  • Desmoplastic
  • Latent period between exposure and development can be up to 40 years
63
Q

What are the presenting symptoms of mesothelioma?

A
  • Progressive SOB
  • Chest pain (diffuse, may affect arm, shoulder, chest wall, abdomen, pleuritic, recurrent pleural effusions
  • Weight loss
  • Anorexia
  • Fever
  • Requires a detailed occupational history
64
Q

What are the signs of mesothelioma?

A
  • General
  • Finger clubbing
  • Cachexia (advanced)
  • Signs of pleural effusion
  • Reduce expansion
  • Tracheal deviation (large effusion)
  • Reduced TVF
  • Stony dull percussion
  • Reduced AE
  • Diminished breath sounds
  • Bronchial breathing above the effusion where the lung is compressed
  • Reduce VR
  • Signs of metastases
  • Lymphadenopathy
  • Hepatomegaly
  • Bone pain/tenderness
  • Abdominal pain/obstruction/ascites
  • Peritoneal malignant mesothelioma
65
Q

What is shown on radiological imaging of mesothelioma?

A
  • Chest x-ray
  • Pleural thickening/effusion
  • Pleural plaque - nodular/irregular pleural shadowing
  • Pleural calcifications
  • Pulmonary fibrosis
  • CT scan
  • Pleural thickening/effusion
  • Discrete pleural masses
  • Multi-loculated effusions
66
Q

What other investigations can be done for patients with mesothelioma?

A
  • USS guided pleural tap
  • Bloody pleural fluid can send for cytology
  • Pleural biopsy
  • With Abram’s needle/CT guided - diagnostic biopsy sent for histology
  • Thorascopic surgical biopsy
  • Only definitive way to get a histolopathological diagnosis as percutaneous pleural biopsy/pleural tap have poor sensitivity
67
Q

What are the conservative approaches to management of mesothelioma?

A
  • Patient education
  • Inform the patient that the prognosis is poor despite current trimodality approach of surgery, radiotherapy and chemotherapy
  • Inform patient regarding the UK Industrial Injuries Act compensation
68
Q

What are the medical options for the management of mesothelioma?

A
  • Chemotherapy
  • Pemetrexed
  • Gemcitabine + Cisplatin (can improve survival although relatively chemoresistant)
  • Radiotherapy
  • Controversial as it is relatively radio-resistant
69
Q

What are the surgical options for the management of mesothelioma?

A
  • Radical surgery indicated for stage I and II with favourable histology - epithiliod in specialist centres
  • VATS (video assisted thorascopic surgery) decortication and pleurodesis
  • Indications - palliative management of symptomatic pleural effusions
70
Q

What is the prognosis of mesothelioma?

A
  • Poor
  • Median survival <1 year - especially if without treatment with Pemetrexed i.e. Alimata which prolongs median survival from 9-12 months
  • Often the diagnosis is made on post mortem
  • > 650 deaths / year in the UK
71
Q

What is a lung abscess?

A
  • Cavitating area of localised suppurative infection within the lung
72
Q

What is empyema?

A
  • Pus in the pleural space
73
Q

What are the causes of lung abscess?

A
  • Inadequately treated pneumonia
  • Aspiration (alcoholism, oesophageal obstruction, bulbar palsy)
  • Bronchial obstruction (tumour, foreign body)
  • Pulmonary infarction
  • Septic emboli (septicaemia, right heart endocarditis, IVDU)
  • Subphrenic/hepatic abscess
74
Q

What are the causes of empyema in the lung?

A
  • Inadequately treated pneumonia
75
Q

What are the symptoms of a lung abscess?

A
  • Swinging fever
  • Cough
  • Sputum (purulent, foul smelling)
  • Pleuritic chest pain
  • Haemoptysis
  • Malaise
  • Weight loss
76
Q

What are the symptoms of a lung empyema?

A
  • Patient with resolving pneumonia develops recurrent fever
77
Q

What are the signs of a patient with a lung abscess?

A
  • Clubbing
  • Anaemia
  • Lung crackles
78
Q

What are the signs in a patient with empyema?

A
  • Features of pleural effusion
79
Q

What are the appropriate blood investigations in a patient with a lung abscess or empyema?

A
  • Blood cultures
  • FBC
  • Hb
  • WCC
  • ESR
  • CRP
80
Q

What are the imaging findings in a patient with a lung abscess vs empyema?

A
  • Chest x-ray
  • Abscess - Wall cavity often with fluid level
  • Empyema - Pleural effusion
81
Q

What other investigations can be done for patients with abscess/empyema?

A
  • Sputum MC&S and cytology - abscess
  • USS guided pleural tap
  • Aspirate pleural fluid (empyema yellow/turbid with pH<7.2, low glucose, high LDH)
  • Bronchoscopy to obtain diagnostic specimens
82
Q

What is the approach to medical management of empyema or lung abscess?

A
  • Intercostal chest drain - preferable under radiological guidance to drain the empyema - adhesions/loculations can make this difficult
  • For postural drainage include repeated aspiration
  • Antibiotics for abscess according to sensitivities - continue for 4-6 weeks
  • Surgical excision / decortication may be required
83
Q

What are the possible risks of an abscess?

A
  • Empyema (20-30%)

Y5 Respiratory System (3 decks)

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