Chronic lung disease

Question 1

What is deemed a significant bronchodilator response?

Answer 1

Increase in FEV1 of 200 mL / 12%

Question 2

Pulmonary fibrosis.
a) Causes
b) Classic features
c) Gold standard investigations (and supporting tests)
d) Management

Answer 2

a) - Idiopathic
- Environmental risks: smoking, coal dust, asbestos, silica, hypersensitivity pneumonitis (e.g. bird fancier)
- Medications: amiodarone, nitrofurantoin, ergot-derived D2-agonists (bromocriptine, cabergoline)
- Connective tissue diseases: RA, SLE, systemic sclerosis and Sjögren’s syndrome
- GORD (higher in patients with IPF)

b) Dyspnoea (progressive, on exertion), dry cough, digital clubbing, diffuse bilateral fine crackles

c) - HRCT: usual interstitial pneumonia (subpleural basal predominance, reticular pattern, honeycombing)
- Pulmonary function tests: spirometry (restrictive pattern), gas transfer (reduced)

d) - Conservative: smoking cessation, pulmonary rehabilitation, annual influenza/pneumococcal vaccines
- Oxygen therapy for hypoxaemia
- PPI for cough

Disease-modifying therapy.
- Perfenidone (antifibrotic)
- Nintedanib (tyrosine-kinase inhibitor)
- Lung transplantation

Question 3

Asbestos-associated lung disease
a) Give the different stages
b) Investigation of choice and classical finding
c) Management (non-malignant)

Answer 3

a) - Benign disease (Pleural plaques, thickening, effusions)
- Interstitial lung disease (asbestosis, a type of pneumoconiosis)
- Malignant disease (mesothelioma and lung Ca)
note: risk increases with degree and duration of exposure)

b) HRCT - honeycombing of the lung. Also, pleural plaques, thickening and effusions

c) - Entitlement to compensation
- Seasonal influenza/pneumococcal vaccination
- Support symptoms

Question 4

Malignant mesothelioma.
a) Presentation - classical? Peritoneal? Metastatic?
b) Investigations
c) Management
d) Prognosis

Answer 4

a) - Pleural: SOB, chest pain, weight loss
- Peritoneal: ascites
- Metastatic: bone pain, hepatomegaly, lymphadenopathy, GI obstruction

b) Imaging - CXR, CT, PET
- Pleural biopsy to confirm diagnosis (US/CT-guided percutaneous biopsy)

c) Surgery: if extremely localised, may be curative
- Chemo: platinum-based, improves survival (non-curative)
- Adjuvant RT

d) Poor - Median survival around 1 year

Question 5

Acute asthma: staging (adults)
a) Moderate
b) Severe - any one of…? (PUFF)
c) Life-threatening - any one of…? (PORSCHE)
d) Near-fatal

• Note: in children, there is no moderate, only acute severe and acute life-threatening (criteria broadly map onto adult acute severe and life-threatening)

Answer 5

a) Increasing symptoms, PEF >50–75% best/predicted, no features of acute severe asthma

b) PEF 33-50% best/predicted,
Unable to talk in complete sentences,
Fast breathing (RR 25+),
Fast heart rate (HR 110+)

c) PEF < 33% best/predicted,
Oxygen low (SpO2 <92% / PaO2 <8 kPa) or CO2 ‘normal’
Reduced consciousness
Silent chest
Cyanosis,
Hypotension
Exhaustion
-Also: arrhythmia

d) T2RF: Raised PaCO2 and/or requiring mechanical ventilation

Question 6

Acute asthma: investigations (adults)
a) What tests should always be performed?
b) Other test in suspected severe asthma?
c) When to do a CXR?

Answer 6

a) SpO2, other obs, peak flow/spirometry

b) ABG (especially if SpO2 < 92% on air)

c) - Suspected pneumothorax,
- pneumonia,
- failure to respond to treatment,
- life-threatening asthma,
- requiring ventilation

Question 7

Acute asthma: admission criteria (adults)
a) Who should be admitted from ED?
b) Who can be discharged from ED?

Answer 7

a) Anyone with life-threatening/near-fatal asthma or severe asthma not responding to treatment

b) Anyone whose peak flow is >75% predicted one hour following treatment (provided no other issues)

Question 8

Acute asthma: treatment (adults)
- O SHIME
- criteria for ITU admission
- Follow up

Answer 8

Oxygen.
- initially 100% oxygen at 15L/min via non-rebreathe mask
- aim for 94 - 98% (may need to titrate, e.g. Venturi)
- ABG if < 92% / risk of hypercapnia/ severe asthma

Salbutamol.
- via spacer: 4 puffs initially, then 2 puffs every 2 minutes up to a maximum 10); or,
~ 5-10 mg/hour via oxygen-driven nebulisers if severe/life-threatening

Steroids.
- Prednisolone oral 40 mg; or,
- Hydrocortisone 100mg IV

Ipratropium.
- 0.5 mg, 4-6 hourly nebs (with salbutamol nebs)

Magnesium sulphate
- IV infusion 1.2 - 2.0g over 20 mins
- in acute severe asthma, not responding to bronchodilator therapy
- beware hypotension!

Escalate to ICU - criteria:
- Anyone with life-threatening/near-fatal asthma who is failing to respond to therapy, i.e. :
• deteriorating PEF
• hypercapnia, or persisting/worsening hypoxia
• acidosis or fall in pH
• exhaustion, feeble respiration
• drowsiness, confusion, altered conscious state
• respiratory arrest

Follow-up
- Notify GP within 24 hours of discharge
- Respiratory specialist appointment

Question 9

Asthma diagnosis.
a) Children under 5
b) Children over 5
c) Adults

Answer 9

a) Watchful waiting or treatment trial (as per asthma stepwise management - SABA + low-dose ICS or LTRA&raquo_space; SABA + low-dose ICS and LTRA&raquo_space; addition of LABA/ theophylline&raquo_space; addition of oral steroid)

b) Diagnostic tests can be used:
- Spirometry to assess for obstructive pattern
- Bronchodilator tests
- Atopic tests - eosinophilia, skin tests, IgE

c) As for children over 5 (but atopic tests less relevant)

Question 10

Advice for managing asthma attacks

Answer 10

1. Sit up straight and keep calm
2. Give one puff of reliever inhaler every 30 - 60 second, up to 10 puffs if needed
3. If no improvement/ you are concerned, call 999
4. If the ambulance hasn’t arrived after 15 minutes, repeat step 2

Question 11

Asthma features.
a) Features increasing likelihood of asthma (WHEEZING)
b) Features decreasing likelihood of asthma

Answer 11

a) Wheeze, cough, dyspnoea, chest tightness (> 1)
History of atopy
Exacerbating factors/triggers (exercise, cold, allergy)
Expiratory wheeze widespread on auscultation
Zzzz (nocturnal symptoms)
Improvement on treatment
Not related to cold symptoms (constant)
Goes up and down (day-to-day and diurnal)

b) - Symptoms only occurring in conjunction with colds;
- no interval symptoms;
- isolated cough without wheeze or breathing difficulties;
- moist cough;
- cardiac symptoms;
- normal respiratory examinations / PFTs;
- no response to asthma treatment.

Question 12

Asthma drug management (adults)

Answer 12

1st line: SABA + low-dose ICS
2nd line: add LABA (usually as combination ICS/LABA inhaler)
3rd line: increase ICS to medium-dose
4th line: trial add-on therapy (e.g. LTRA, theophylline, LAMA) or increase ICS to high hose / trial MART
5th line: oral prednisolone (lowest effective dose)

Question 13

Asthma drug management (children)

Answer 13

1st line: SABA + very low-dose ICS or LTRA (< 5 yrs)
2nd line: … + very low-dose ICS and LTRA (< 5) or LABA (> 5)
3rd line: increase ICS to low dose (remove LABA if no response)
4th line: trial add-on therapy (LTRA if not already; or theophylline SR) or increase ICS to medium dose / trial MART
6th line: oral prednisolone tablet (lowest effective dose)

Differences to adults:
- LRTA may be tried at step 1 or 2
- Start with VERY low-dose ICS (step 1), increase to low dose (step 3), and possibly to medium dose (step 4)

Question 14

Asthma: non-drug management

Answer 14

• Annual asthma review: check symptoms, check compliance, review medications, check inhaler technique, record peak flow, review asthma action plan, etc.
• Avoid triggers - house dust mite, pets, etc.
• Asthma action plan
• Peak flow diaries
• Smoking cessation
• Weight reduction
• Immunisations

Question 15

MART therapy.
a) What is it?
b) Who should be considered for it?

Answer 15

a) Maintenance and reliever therapy (MART): reliever and preventer inhaler in one device.
- To be taken regularly every day
- Dose increased when symptoms are worse

b) People still experiencing symptoms on preventer inhaler and who have tried alternative treatments like a LTRA

Question 16

Asthma review.
a) 3 core questions for symptoms (Note: 1 ‘yes’ indicates medium morbidity and 2 or 3 indicate high morbidity)
b) 2 additional questions to assess risk
c) Other things to perform at review

Answer 16

a) - In the last month/week have you had difficulty sleeping due to your asthma (including cough)?
- Have you had your usual asthma symptoms (eg, cough, wheeze, chest tightness, SOB) during the day?
- Has your asthma interfered with your usual daily activities (eg, school, work, housework)?
(also: how often have you needed to use your reliever)

b) - Have you been admitted for asthma in the last year?” - “Have you ever needed ITU care for asthma?”

c) Check medication compliance, review medications, check inhaler technique, record peak flow, review asthma action plan, smoking cessation advice, weight management, etc.

Question 17

COPD: aetiology
a) Pathogenesis
b) Risk factors

Answer 17

a) - Chronic inflammation of the airways (chronic bronchitis) in response to noxious particles (particularly cigarette smoke)
- Leading to progressive alveolar damage (emphysema) and poorly reversible airway obstruction

b) Cigarette smoking; other noxious particles - air pollution, marijuana smoking, occupational exposure to dusts and chemicals, alpha-1-antitrypsin deficiency, low socioeconomic status, asthma (may overlap)

Question 18

COPD: diagnosis
a) Consider in who?
b) Gold standard COPD diagnostic tool
c) FEV1:FVC ratio in obstructive disease
d) COPD staging
e) What other investigations should be performed as part of the workup?

Answer 18

a) Patients >35 years with a risk factor (eg smoking), presenting with SOBOE, chronic cough, regular sputum production, frequent winter ‘bronchitis’ or wheeze.

b) Post-bronchodilator spirometry

c) < 0.7 (in COPD, this should be present after bronchodilator use also - not fully reversible)

d) Based on FEV1:
- Stage 1: mild - FEV1 is >80% predicted.
- Stage 2: moderate - FEV1 is 50-79% predicted.
- Stage 3: severe - FEV1 is 30-49% predicted.
- Stage 4: very severe - FEV1 is below 30% predicted (or FEV1 less than 50% but with respiratory failure).

e) - CXR - exclude other pathology (e.g. Lung Ca)
- Height and weight for BMI calculation
- FBC - identify anaemia/polycythaemia

Question 19

MRC dyspnoea scale

Answer 19

• Grade 1: only SOB on strenuous exertion (normal)
• Grade 2: SOB when hurrying on level ground or walking up a slight incline
• Grade 3: walks slower than others because of SOB
• Grade 4: stops for breath after walking ~100 metres/ for a few minutes on level ground
• Grade 5: too breathless to leave the house or breathless on dressing/undressing

Question 20

Management of stable COPD.
a) Conservative measures
b) Drug management (standard: steps 1 - 4 …+ adjuvants)
c) Other interventions
d) Monitoring
e) Role of prophylactic antibiotics
f) Oxygen therapy: indications and aims

Answer 20

a) Smoking cessation, pulmonary rehabilitation, patient education, develop a self-management plan, weight loss and nutritional support, optimise treatment of comorbidities

b) - Only start inhaled therapies in symptomatic patients
- Step 1: SABA/SAMA as required
- Step 2a (if ACO features): LABA + ICS
- Step 2b (if no ACO features): LABA + LAMA
- Step 3: LABA + LAMA + ICS
- Step 4: oral steroids/ theophylline
- Adjuvants: mucolytics (e.g. carbocysteine), oxygen, CPAP/BiPAP

c) - Annual influenza and pneumococcal vaccine
- Backup medication (pred / Abx)

d) Spirometry, check compliance with medication, inhaler technique, enquire about exacerbations, evaluate need for stepping up management (e.g. oxygen),

e) - Patients with frequent severe exacerbations - usually azithromycin (check ECG and LFTs first)
- Do sputum culture for targeted therapy

f) - Patients with severe chronic hypoxaemia: PaO2 <7.3 kPa or <8 kPa if pul HTN/cor pulmonale/polycythaemia
- NOT IN SMOKERS
- Give at no more than 28% via Venturi (4L/min) or 2L/min via nasal cannulae
- Aim for SaO2 88-92%,

Question 21

Management of acute exacerbations of COPD.
a) Define acute exacerbation
b) Non-bacterial - symptoms, management
c) Bacterial - symptoms, management

Answer 21

a) Acute worsening of the patient’s symptoms, beyond normal day-to-day variations (usually caused by virus, sometimes bacteria)

b) - Increased SOB, dry cough or wheeze
- Increase frequency of short-acting bronchodilators (SABA/SAMA)
- 7 - 14 days oral 30mg prednisolone six times daily (osteoporosis prophylaxis in patients requiring frequent courses)

c) Sputum changes: increased volume, purulence or thickness; signs of CAP (consolidation, fever, etc.)
- Consider sputum culture
- If given - amoxicillin/doxy/clari for 5/7

Question 22

Known COPD patient presents increasing SOBOE, waking at night breathless, ankle swelling and fatigue.
a) What might have occurred? - give the pathogenesis
b) If weight loss and haemoptysis were present, what would you be worried about? - management?

Answer 22

a) Cor pulmonale: chronic hypoxaemia causes pulmonary hypertension, leading to RVH and eventually right heart failure, progressing to CCF

b) Lung Ca - 2 week wait referral and urgent CXR

Question 23

Asthma vs COPD.
a) Clinical features
b) Patient demographics
c) Testing

Answer 23

a) - Variability in symptoms: variable in people with asthma; persistent and progressive in COPD.
- Nocturnal symptoms: more common in asthma.
- Presence of persisting productive cough: common in COPD but not in asthma

b) - Age at onset: usually younger in asthma.
- Smoking history: in most with COPD.

c) - Large (>400 ml) response to bronchodilators.
- Large (>400 ml) response to 30 mg daily pred for 2/52
- Serial peak flow measurements showing 20% or greater diurnal or day-to-day variability.

Question 24

Asthma/COPD overlap syndrome (ACO)
a) Define
b) Management

Answer 24

a) Patients with COPD who have:
- Prior diagnosis of asthma or atopy
- Raised blood eosinophil count
- Substantial variation in FEV1 over time (at least 400 ml) - Substantial diurnal variation in peak flow (at least 20%)

Patients with asthma who:
- Smoke and develop non-fully reversible airways obstruction

b) ACO features - suggests ICS responsiveness - step 2 of COPD management advises ICS + LABA

Question 25

Examples of the following classes:
a) SABA (2)
b) SAMA
c) LABA (2)
d) LAMA
e) ICS (3)
f) Mucolytic
g) LTRA

Answer 25

a) Salbutamol, terbutaline

b) Ipratropium

c) Formoterol, salmeterol

d) Tiotropium, glycopyrronium bromide

e) Beclometasone, budesonide, fluticasone

f) Carbocysteine

g) Montelukast

Question 26

Pulmonary hypertension.
a) Define
b) Aetiology
c) Clinical features
d) Investigations
e) Management of PH

Answer 26

a) Mean PAP ≥25 mm Hg at rest as assessed by right heart catheterisation

b) - Idiopathic primary PH (rare)
- Respiratory disease: chronic hypoxia (COPD, lung fibrosis, chronic exposure to high altitude, OSA) or chronic hypercapnia (hypoventilation)
- Connective tissue disease: CREST, Sjogren’s, vasculitis
- Left heart disease: CCF, mitral stenosis/regurg, congenital heart disease (Eisenmenger’s)
- Other: HIV, portal hypertension, drug-induced, thyroid disease, haematological causes

c) Progressive SOB, weakness and tiredness;
- Later: RV failure (oedema ascites, raised JVP)

d) - Bloods: FBC (anaemia, polycythaemia), BNP, LFTs, antibodies (connective tissue disease)
- Respiratory function tests: spirometry/ gas transfer
- Imaging: CXR/HRCT, ECHO
- Special tests: right heart catheterisation to confirm Dx

e) - Manage underlying cause
- Cardiosupportive therapy: diuretics, oxygen, ?digoxin
- Pulmonary vasodilators: PDE-5 inhibitors (sildenafil), endothelin-A inhibitors (bosentan/ ambrisentan), intravenous prostacyclin analogues
- Lung transplantation

Question 27

Cor pulmonale.
a) Pathogenesis
b) Causes - acute and chronic
c) Clinical features
d) Management

Answer 27

a) Lung disease leading to increased pulmonary arterial pressure and subsequently to RV failure

b) Chronic:
- Parenchymal - COPD (main cause), fibrosis, CF
- Chronic hypoventilation - NMD
- Vascular - vasculitis, recurrent PE, sickle cell, primary PH
Acute - PE, AECOPD

c) Worsening SOB, fatigue, leg swelling

d) - Respiratory function tests: spirometry/ gas transfer
- Bloods: FBC (anaemia, polycythaemia, infection), BNP (heart failure), blood gas (resp failure)
- Imaging: CXR, ECHO,
- Special tests: ?right heart catheterisation
- Manage underlying cause
- Diuretics
- Oxygen therapy (if hypoxic)
- Lung transplantation
- Smoking cessation

Question 28

CF: pathogenesis
a) Genetics
b) Pathophysiology
c) Clinical features (including signs on examination)
d) Diagnosis
- most commonly when?
- if suspected otherwise - test in children/ adults? (How many? Reasons for false positives?)

Answer 28

a) Autosomal recessive: CFTR gene on Ch 7 - most common mutation is delta-F508

b) Inadequate chloride transport leads to dehydrated luminal secretions, affecting any luminal tract (airways, GI, biliary, pancreatic, reproductive)

c) - Lungs: recurrent infection, nasal polyps, SOB, chronic wet cough, chronic sinusitis
- Pancreas: pancreatitis, diabetes
- GI: meconium ileus, diarrhoea, malabsorption, FTT, rectal prolapse, DIOS
- Biliary: jaundice, gallstones and cholecystitis
- Sexual: male infertility (congenital absence of vas)
- Signs: clubbing, crackles, wheeze, low FEV1

d) - Newborn heel prick - immunoreactive trypsinogen (IRT)
- Antenatally (CVS, amniocentesis)
- Sweat test for high chloride (2 at different times) - false positives in Addisons, glycogen storage disease, atopic eczema, hypothyroid)
- CFTR gene testing

Question 29

CF: management
a) Respiratory
b) Gastrointestinal
c) Other
d) Monitoring tests to be done at regular intervals

Answer 29

a) - Pulmonary function testing, sputum cultures, CXR/CT as necessary
- Exercise
- Separation of CF patients
- Chest physiotherapy (for secretions)
- Bronchodilators
- Nebulised saline (osmotic effect)
- Dornase alfa (mucolytic)
- Prophylactic antibiotics (for staph, pseudomonas, moraxella)

b) - Monitor weight, glucose/HbA1c, liver function (USS)
- High calorie, high fat diet
- Vitamin supplements (ADEK)
- Pancreatic enzymes (Creon)
- Ursodeoxycholic acid (for bile stasis)

c) - Psychological support,
- Genetic counselling: males (infertile), females (carriage of CFTR gene)

d) - Pulmonary function tests. CXR. Respiratory cultures.
- USS liver. Vitamin D levels. Blood glucose.
- DEXA scan.

Question 30

Bronchiectasis.
a) Pathogenesis
b) Causes
c) Presentation
d) Investigations
e) Management

Answer 30

a) Permanent dilatation AND thickening of the airways WITH excessive sputum production AND subsequent bacterial colonisation and recurrent infections

b) Idiopathic, post-infective (childhood measles, influenza, pertussis, RSV; pneumonia, TB), HIV, connective tissue disease (RA, SLE, CREST), chronic lung disease (asthma, COPD), congenital (CF, Kartagener’s)

c) - Chronic wet cough, excessive sputum production, recurrent infections; other - CP, SOB, haemoptysis
- Coarse crackles, wheeze, clubbing

d) - Bedside: spirometry, Sputum culture
- Bloods: FBC, CRP
- Imaging: CXR, HRCT (gold standard - bronchial dilatation)
- Special tests: ?CF gene test, ?bronchoscopy

e) - Monitor lung function, sputum cultures etc.
- Conservative: smoking cessation, exercise, healthy diet, immunisations (influenza, pneumococcus)
- Secretions: chest physiotherapy, hypertonic saline, dornase alfa
- Lung function: bronchodilators
- Prophylactic ABx if recurrent infections (3+ per year)
- Acute exacerbation: sputum culture, 1st line empirical ABx (amox/doxy/clari) then narrow-spectrum based on sensitivities
- Oxygen therapy
- Lung transplantation

Question 31

Occupational asthma
a) Most commonly affected workers
b) Screening question
c) Diagnosis
d) Management

Answer 31

a) Bakers, farmers, waiters, cleaners, painters, food processors, forestry workers, chemical workers, plastics and rubber workers, metal workers, welders, textile workers, electrical production workers, storage workers, dental workers and lab technicians

b) Do symptoms improve away from work/during the holidays? (Or are they worse while at work?)

c) Serial peak flow measurements (at least 4 per day), both at work and away from work

d) Identify the cause, remove them from exposure, and provide them with worthwhile alternative employment