Respiratory Flashcards
1
Q
What are some environmental triggers for asthma?
A
Allergens (pollen, animal dander, dust mites)
Respiratory infections (e.g., viral infections)
Tobacco smoke
Air pollution
Occupational exposures (chemical irritants, industrial dust).
2
Q
What are the main risk factors for developing asthma?
A
Personal/family history of atopy
Maternal smoking (during pregnancy and around child)
Low birth weight
Not being breastfed
Exposure to high concentrations of allergens
Air pollution
Hygiene hypothesis: reduced exposure to infections in childhood, leading to a Th2-dominant immune response.
3
Q
What other atopic conditions are commonly associated with asthma?
A
Atopic dermatitis (eczema) and allergic rhinitis (hay fever).
4
Q
How is asthma defined?
A
Asthma is a chronic inflammatory disorder of the airways secondary to type 1 hypersensitivity, characterized by reversible bronchospasm causing airway obstruction.
5
Q
What causes bronchoconstriction in asthma?
A
Contraction of airway smooth muscle triggered by inflammatory mediators such as histamine and leukotrienes.
6
Q
What is airway remodeling in asthma?
A
Chronic inflammation leads to structural changes in the airway wall, such as:
Subepithelial fibrosis
Increased smooth muscle mass
Mucus gland hypertrophy
Angiogenesis
7
Q
What are the common symptoms of asthma?
A
–> Wheezing: A high-pitched, whistling sound during expiration due to narrowed airways.
–> Cough: Dry or productive, often worse at night or early morning, triggered by allergens, cold air, exercise, or respiratory infections.
–> Dyspnoea (shortness of breath): Caused by bronchoconstriction, airway inflammation, and mucus production, experienced during physical exertion or at rest.
–> Chest tightness: A sensation caused by airway obstruction and increased work of breathing, often accompanied by coughing and shortness of breath.
8
Q
What objective tests are used to diagnose asthma in patients ≥ 5 years old according to NICE 2017 guidelines?
A
Eosinophil count - raised
FeNO (Fractional Exhaled Nitric Oxide):
Adults: Positive if FeNO ≥ 40 parts per billion (ppb).
Children: Positive if FeNO ≥ 35 parts per billion (ppb).
Spirometry:
Obstruction is indicated by an FEV1/FVC ratio < 70% or below the lower limit of normal.
Bronchodilator Reversibility (BDR) Test:
Adults: Positive if FEV1 improves by ≥ 12% and volume increases by 200 ml or more.
Children: Positive if FEV1 improves by ≥ 12%.
Bronchial challenge test
9
Q
What are the key steps in managing asthma according to NICE 2017?
A
- Newly diagnosed asthma:
SABA as needed. - Not controlled on Step 1 / symptoms ≥ 3/week:
SABA + low-dose ICS. - Not controlled on Step 2:
SABA + low-dose ICS + LTRA. - Not controlled on Step 3:
SABA + low-dose ICS + LABA.
Continue LTRA based on response.
- Not controlled on Step 4:
Switch to MART (low-dose ICS/LABA). - Not controlled on Step 5:
Medium-dose ICS MART or return to fixed-dose ICS + LABA. - Not controlled on Step 6:
High-dose ICS or add a new drug (e.g., LAMA, theophylline).
Seek specialist advice if needed.
MART:
Combined ICS/LABA inhaler for daily and relief use (only with fast-acting LABA).
Inhaled Corticosteroid Doses (Adults):
Low: ≤ 400 mcg budesonide
Moderate: 400-800 mcg
High: > 800 mcg
10
Q
How are acute asthma exacerbations classified according to NICE?
A
Moderate Acute Asthma Exacerbation:
Symptoms: Increasing.
PEFR: >50-75% of best/predicted.
Other: No severe features.
Acute Severe Asthma Exacerbation:
PEFR: 33-50% of best/predicted.
RR: ≥ 25 breaths/min.
HR: ≥ 110 beats/min.
Other: Unable to complete sentences in one breath.
Life-Threatening Asthma Exacerbation:
PEFR: <33% of best/predicted.
SpO₂: <92%.
PaO₂: <8kPa.
Other: Silent chest, cyanosis, new arrhythmia, exhaustion, reduced GCS, hypotension.
Near-Fatal Asthma Exacerbation:
PaCO₂: >6kPa (raised) and/or mechanical ventilation required.
11
Q
What are the common triggers and symptoms of acute asthma exacerbations?
A
Triggers:
Viruses (e.g., rhinovirus)
Bacteria
Allergens (e.g., mold, pet dander)
Tobacco smoke
Symptoms:
Progressive breathlessness
Cough
Wheeze
Chest tightness
Signs of Severe Exacerbation:
Tachypnoea
Tachycardia
Inability to speak in full sentences
Silent chest
12
Q
What investigations are recommended for managing acute asthma exacerbations?
A
PEFR / FEV1
Assesses severity of exacerbation.
PEFR is preferred in acute settings; expressed as a percentage of the patient’s best/predicted score.
Predicted score is based on age and height if best score is unavailable.
SpO₂ Measurement
Normal saturation: >94%.
<92% indicates life-threatening exacerbation requiring urgent treatment and increased risk of hypercapnia.
Pulse oximetry is unreliable in shock or anaemic patients.
Arterial Blood Gas (ABG)
Indicated if SpO₂ <92% or PEFR ≤30% of best/predicted.
Measures PaO₂ and PaCO₂; lower PaO₂ correlates with obstruction severity.
Hypercapnia (PaCO₂ >6kPa) suggests near-fatal attack, correlating with FEV1 around 20% of predicted.
Most patients exhibit respiratory alkalosis; hypercapnia can lead to acidosis.
Venous blood gas may be used if ABG is not possible, though it’s less useful.
Chest X-ray
Not generally required unless another diagnosis (e.g., pneumonia) is suspected.
13
Q
What are some differential diagnoses to consider in patients presenting with severe acute asthma exacerbation?
A
–> Acute Exacerbation of COPD
More likely diagnosed later in life, often with a history of smoking (≥20 pack-years).
Lacks diurnal variation and day-to-day worsening typical in asthma.
–> Pneumothorax
Similar presentation but often causes pleuritic pain.
Chest X-ray is essential if pneumothorax is suspected.
–> Foreign Body Aspiration
May cause wheezing with more acute onset.
Differentiated by chest X-ray to identify radiopaque structures.
–> Vocal Cord Dysfunction
Presents with dyspnoea and stridor; can be difficult to differentiate from asthma.
Evidence may be found on video laryngostroboscopy.
–> Pulmonary Embolism
Usually presents more acutely than asthma exacerbation.
Commonly associated with pleuritic pain and haemoptysis, which are not typical in asthma.
14
Q
What is the management protocol for patients with near-fatal or life-threatening acute asthma?
A
–> Admission to Hospital
Immediate admission for near-fatal or life-threatening exacerbations.
Admission for severe cases failing to respond to initial treatment.
–> Oxygen Therapy
Start supplemental oxygen if hypoxaemic.
Administer 15L via non-rebreather mask; titrate to maintain SpO₂ 94-98%.
Consider nasal cannulae or Venturi masks.
Initial Treatment
–> High-dose inhaled SABA (e.g., salbutamol, terbutaline).
Standard pressurised metered-dose inhaler (pMDI) or oxygen-driven nebulizer for non-life-threatening cases.
Nebulised beta₂-agonists for life-threatening cases.
–> Corticosteroids
Administer 40-50mg prednisolone orally daily for at least 5 days.
–> Additional Treatments
Nebulised ipratropium bromide (0.5mg every 4-6 hours) for severe cases.
Consider magnesium sulfate (IV or nebulized) and/or intravenous aminophylline if no response to initial treatments.
–> Mechanical Ventilation
Considered for coma, arrest, severe fatigue, or cardiovascular compromise.
Consult senior physicians and anaesthesia for guidance.
Post-Exacerbation Review
identify possible triggers for the attack.
Review inhaler use and technique.
Optimize treatment and develop a plan for preventing further exacerbations.
15
Q
What are the key features of Chronic Obstructive Pulmonary Disease (COPD)?
A
Cough: Often productive
Dyspnoea: Difficulty breathing
Wheeze: Whistling sound during breathing
Right-sided Heart Failure: May develop in severe cases, resulting in peripheral oedema
16
Q
What investigations are recommended for suspected Chronic Obstructive Pulmonary Disease (COPD)?
A
–> Post-Bronchodilator Spirometry:
FEV1/FVC ratio < 0.7 indicates airflow obstruction
–> Chest X-ray:
Assess for hyperinflation, bullae (can mimic pneumothorax), and flat hemidiaphragm
Exclude lung cancer
–> Full Blood Count:
Exclude secondary polycythaemia
–> Body Mass Index (BMI) Calculation:
Assess nutritional status
17
Q
What factors should be considered for diagnosing COPD according to NICE?
A
Age: Patients over 35 years
Smoking History: Current or ex-smokers
Symptoms:
Exertional breathlessness
Chronic cough
Regular sputum production
18
Q
How is the severity of COPD categorized based on FEV1?
A
Stage 1 - Mild: FEV1/FVC < 0.7 with FEV1 > 80% predicted; symptoms should be present to diagnose COPD.
Stage 2 - Moderate: FEV1/FVC < 0.7 with FEV1 50-79% predicted.
Stage 3 - Severe: FEV1/FVC < 0.7 with FEV1 30-49% predicted.
Stage 4 - Very Severe: FEV1/FVC < 0.7 with FEV1 < 30% predicted.
19
Q
What criteria should be assessed to determine if a COPD patient is eligible for long-term oxygen therapy (LTOT)?
A
Very severe airflow obstruction (FEV1 < 30% predicted)
Severe airflow obstruction (FEV1 30-49% predicted) may also be considered
Cyanosis
Polycythaemia
Peripheral oedema
Raised jugular venous pressure
Oxygen saturations ≤ 92% on room air
20
Q
What pO2 levels qualify a COPD patient for long-term oxygen therapy (LTOT)?
A
pO2 < 7.3 kPa
pO2 between 7.3 - 8 kPa if they also have:
Secondary polycythaemia
Peripheral oedema
Pulmonary hypertension
21
Q
What are the key components of general management for COPD according to the 2018 NICE guidelines?
A
Smoking cessation advice, offering nicotine replacement therapy, varenicline, or bupropion
Annual influenza vaccination
One-off pneumococcal vaccination
Pulmonary rehabilitation for those functionally disabled by COPD (usually MRC grade 3 and above)
22
Q
What is the first-line bronchodilator therapy for COPD patients according to NICE?
A
A short-acting beta2-agonist (SABA) or short-acting muscarinic antagonist (SAMA) is the first-line treatment.
23
Q
How does NICE recommend determining if a COPD patient has asthmatic or steroid-responsive features?
A
Previous, secure diagnosis of asthma or atopy
Higher blood eosinophil count
Substantial variation in FEV1 over time (≥ 400 ml)
Substantial diurnal variation in peak expiratory flow (≥ 20%)
24
Q
What bronchodilator therapy is recommended for COPD patients without asthmatic or steroid-responsive features?
A
Add a long-acting beta2-agonist (LABA) + long-acting muscarinic antagonist (LAMA). If already using a SAMA, switch to a SABA.
25
What therapy is recommended for COPD patients with asthmatic or steroid-responsive features?
LABA + inhaled corticosteroid (ICS)
If symptoms persist, offer triple therapy (LAMA + LABA + ICS)
If already using a SAMA, switch to a SABA
26
When is oral theophylline recommended in COPD management?
Oral theophylline is recommended after trials of short and long-acting bronchodilators or for patients who cannot use inhaled therapy. The dose should be reduced if macrolide or fluoroquinolone antibiotics are co-prescribed.
27
Under what circumstances does NICE recommend oral prophylactic azithromycin for COPD patients?
Azithromycin prophylaxis is recommended if patients:
Do not smoke
Have optimised standard treatments
Continue to have exacerbations
Have had a CT thorax to exclude bronchiectasis and sputum culture to exclude atypical infections and tuberculosis
Have normal LFTs and an ECG to exclude QT prolongation
28
What criteria must be met for the use of phosphodiesterase-4 (PDE-4) inhibitors in COPD?
NICE recommends PDE-4 inhibitors like roflumilast for patients with:
Severe COPD (FEV1 < 50% predicted normal)
Two or more exacerbations in the last 12 months despite triple therapy with LAMA + LABA + ICS
29
What features indicate cor pulmonale in COPD patients, and how should it be managed?
Features: peripheral oedema, raised jugular venous pressure, systolic parasternal heave, loud P2. Management includes loop diuretics for oedema and considering long-term oxygen therapy. ACE inhibitors, calcium channel blockers, and alpha blockers are not recommended.
30
What are the key factors that improve survival in COPD patients?
Smoking cessation (most important for smokers)
Long-term oxygen therapy (LTOT) in eligible patients
Lung volume reduction surgery in selected patients
31
What are the most common bacterial causes of acute COPD exacerbations?
Haemophilus influenzae (most common cause)
Streptococcus pneumoniae
Moraxella catarrhalis
32
What respiratory viruses are commonly associated with COPD exacerbations?
Respiratory viruses account for around 30% of exacerbations
Human rhinovirus is the most important pathogen
33
What are the clinical features of an acute COPD exacerbation?
Increase in dyspnoea, cough, and wheeze
Increased sputum production, suggestive of an infective cause
Hypoxia and acute confusion in severe cases
34
What is the NICE recommendation for treating an acute exacerbation of COPD?
Increase the frequency of bronchodilator use (consider nebulisers)
Give prednisolone 30 mg daily for 5 days
Use antibiotics if sputum is purulent or there are signs of pneumonia (first-line options: amoxicillin, clarithromycin, doxycycline)
35
When do the NICE guidelines recommend hospital admission for a COPD exacerbation?
Severe breathlessness
Acute confusion or impaired consciousness
Cyanosis
Oxygen saturation < 90%
Social reasons (e.g., inability to cope at home)
Significant comorbidity (e.g., cardiac disease or insulin-dependent diabetes)
36
What oxygen saturation target should be aimed for in COPD patients with risk of hypercapnia during an exacerbation?
An initial oxygen saturation target of 88-92% should be used.
37
What is the recommended oxygen therapy for COPD exacerbations before blood gas results are available?
Use a 28% Venturi mask at 4 l/min and aim for oxygen saturation of 88-92% in patients with risk factors for hypercapnia but no prior history of respiratory acidosis.
38
What are the typical bronchodilator treatments for COPD exacerbations?
Beta adrenergic agonists (e.g., salbutamol)
Muscarinic antagonists (e.g., ipratropium)
39
What corticosteroid therapies are used during severe exacerbations of COPD?
Prednisolone 30 mg orally for 5 days
Intravenous hydrocortisone may be considered in certain cases
40
When is intravenous theophylline considered for COPD exacerbations?
It may be considered in patients who do not respond to nebulised bronchodilators.
41
What type of respiratory failure can COPD patients develop during an exacerbation, and what therapy is often used?
Type 2 respiratory failure may develop, and non-invasive ventilation (NIV) is typically used, especially for respiratory acidosis (pH 7.25-7.35).
42
What is acute bronchitis?
Acute bronchitis is a self-limiting chest infection caused by inflammation of the trachea and bronchi, leading to sputum production and airway swelling.
43
What is the typical duration and cause of acute bronchitis?
Acute bronchitis usually resolves within 3 weeks, but 25% of patients may still have a cough after that. Viral infections are the main cause, especially in autumn and winter (80% of cases).
44
What is the presentation of acute bronchitis?
Patients typically present with an acute onset of:
--> cough: may or may not be productive
--> sore throat
--> rhinorrhoea
--> wheeze
The majority of patients with have a normal chest examination, however, some patients may present with:
--> Low-grade fever
--> Wheeze
45
What are the investigations for acute bronchitis?
acute bronchitis is typically a clinical diagnosis
however, if CRP testing is available this may be used to guide whether antibiotic therapy is indicated
46
How can acute bronchitis be differentiated from pneumonia based on history and examination?
In acute bronchitis, sputum, wheeze, and breathlessness may be absent, while in pneumonia, at least one is usually present.
Acute bronchitis typically lacks focal chest
signs (like dullness to percussion, crepitations, or bronchial breathing) seen in pneumonia.
Additionally, systemic features such as malaise, myalgia, and fever are often absent in acute bronchitis but common in pneumonia.
47
What is the recommended management for acute bronchitis?
Management includes analgesia, good fluid intake, and consideration of antibiotics if the patient is systemically unwell, has pre-existing comorbidities, or a CRP level of 20-100 mg/L (offer delayed antibiotics) or >100 mg/L (offer antibiotics immediately). Doxycycline is first-line (not for children or pregnant women), with alternatives like amoxicillin.
48
What lung diseases can asbestos exposure cause?
Asbestos exposure can lead to a variety of lung diseases, ranging from benign pleural plaques to malignant mesothelioma. Other conditions include pleural thickening, asbestosis, and lung cancer.
49
What are pleural plaques, and what is their significance in asbestos exposure?
Pleural plaques are benign and do not undergo malignant change. They are the most common form of asbestos-related lung disease, typically occurring after a latent period of 20-40 years, and do not require follow-up.
50
What is pleural thickening in relation to asbestos exposure?
Pleural thickening is a condition caused by asbestos exposure that may resemble changes seen after empyema or haemothorax. The exact pathophysiology is not fully understood.
51
What is asbestosis, and how is its severity related to asbestos exposure?
Asbestosis is a lung disease caused by prolonged asbestos exposure, typically with a latent period of 15-30 years. Its severity is related to the length of exposure, leading to lower lobe fibrosis.
52
What are the features of asbestosis?
Features include dyspnoea, reduced exercise tolerance, clubbing, bilateral end-inspiratory crackles, and lung function tests showing a restrictive pattern with reduced gas transfer. It is treated conservatively, as no interventions offer significant benefit.
53
What is mesothelioma, and what are its symptoms?
Mesothelioma is a malignant disease of the pleura caused by asbestos exposure, particularly crocidolite (blue asbestos). It presents with progressive shortness of breath, chest pain, and pleural effusion.
54
What is the prognosis for patients with mesothelioma?
Patients with mesothelioma are typically offered palliative chemotherapy, with limited roles for surgery and radiotherapy. The prognosis is poor, with a median survival of 8-14 months from diagnosis.
55
How does asbestos exposure relate to lung cancer?
Lung cancer is the most common cancer associated with asbestos exposure, with a synergistic effect with cigarette smoke. Smoking cessation is crucial, as the risk of lung cancer is significantly higher in smokers with a history of asbestos exposure
56
What is bronchiectasis, and what are its primary effects?
Bronchiectasis involves permanent dilation of the bronchi, leading to sputum accumulation, chronic cough, continuous sputum production, and recurrent infections due to organisms growing in the wide tubes.
57
What are potential causes of bronchiectasis?
Idiopathic
post infection (Pneumonia)
Whooping cough (pertussis)
Tuberculosis
Alpha-1-antitrypsin deficiency
Connective tissue disorders (e.g., rheumatoid arthritis)
Cystic fibrosis
Yellow nail syndrome
58
What is the significance of yellow nail syndrome in relation to bronchiectasis?
Yellow nail syndrome is characterized by yellow fingernails, bronchiectasis, and lymphoedema. Patients are stable with good clinical signs, making it notable in exams, though it is rare.
59
What are the key presenting symptoms of bronchiectasis?
Shortness of breath
Chronic productive cough
Recurrent chest infections
Weight loss
Haemoptysis
60
What signs may be observed during the examination of a patient with bronchiectasis?
Sputum pot by the bedside
Oxygen therapy (if needed)
Weight loss (cachexia)
Finger clubbing
Signs of cor pulmonale (e.g., raised JVP and peripheral oedema)
Scattered crackles and wheezes in the chest
61
What investigations are used to diagnose bronchiectasis?
Sputum culture to identify organisms (most common: Haemophilus influenzae, Pseudomonas aeruginosa)
Chest x-ray for tram-track opacities and ring shadows
High-resolution CT (HRCT) is the test of choice for diagnosis.
62
What is the general management approach for bronchiectasis?
Vaccines (e.g., pneumococcal, influenza)
Respiratory physiotherapy
Pulmonary rehabilitation
Long-term antibiotics (e.g., azithromycin) for frequent exacerbations
Inhaled colistin for Pseudomonas colonization
Long-acting bronchodilators for breathlessness
Long-term oxygen therapy for low oxygen saturation
Surgical lung resection for specific disease areas
Lung transplant for end-stage disease
63
What is cystic fibrosis (CF), and what causes it?
Cystic fibrosis is an autosomal recessive genetic condition caused by a mutation in the cystic fibrosis transmembrane conductance regulator gene on chromosome 7, most commonly the delta-F508 mutation. It affects mucus glands and leads to thick secretions.
64
What are the key consequences of the cystic fibrosis mutation?
--> Thick pancreatic/biliary secretions causing blockage and lack of digestive enzymes - billiary ducts blocked leading to liver diseases
--> Low volume thick airway secretions leading to bacterial colonization and infections which lead to progressive lung damage (bronchiectasis)
--> Congenital bilateral absence of the vas deferens in males, resulting in infertility
65
What is the typical presentation of cystic fibrosis?
CF is screened at birth with a blood spot test. Meconium ileus, characterized by thick, sticky meconium causing bowel obstruction, is often the first sign. Later signs include recurrent respiratory infections, failure to thrive, and gastrointestinal symptoms.
66
What are common symptoms of cystic fibrosis?
Chronic cough
Thick sputum production
Recurrent respiratory tract infections
Loose, greasy stools (steatorrhoea)
Abdominal pain and bloating
Salty-tasting skin
Poor weight and height gain (failure to thrive)
67
What signs may indicate cystic fibrosis?
Low weight or height on growth charts
Nasal polyps
Finger clubbing
Crackles and wheezes on auscultation
Abdominal distention
68
How is cystic fibrosis diagnosed?
Newborn blood spot testing
Sweat test (gold standard, chloride concentration >60mmol/L)
Genetic testing for CFTR gene
69
What is the sweat test, and why is it important?
The sweat test measures chloride concentration in sweat. It involves inducing sweating with pilocarpine and testing the sweat for chloride levels. A concentration over 60mmol/L confirms cystic fibrosis.
70
What are common microbial colonisers in cystic fibrosis patients?
Staphylococcus aureus
Pseudomonas aeruginosa
Haemophilus influenzae
Klebsiella pneumoniae
Escherichia coli
Burkholderia cepacia
71
How is pseudomonas aeruginosa managed in cystic fibrosis?
Pseudomonas colonization leads to increased morbidity. Management includes long-term nebulized antibiotics like tobramycin and oral ciprofloxacin.
72
What are the key management strategies for cystic fibrosis?
Chest physiotherapy and exercise
High-calorie diet
CREON tablets for pancreatic insufficiency
Prophylactic flucloxacillin
Treatment of chest infections
Bronchodilators (e.g., salbutamol)
Nebulized DNase and hypertonic saline
Regular vaccinations
73
What are additional treatment options for cystic fibrosis?
Lung transplantation for end-stage respiratory failure
Liver transplantation for liver failure
Fertility treatments for male infertility
Genetic counseling
74
What monitoring is required for cystic fibrosis patients?
Patients need regular monitoring for bacterial colonization, diabetes, osteoporosis, vitamin D deficiency, and liver failure, typically in specialist clinics every 6 months.
75
What are the key screening and diagnostic tests for cystic fibrosis, and how are they monitored
--> Newborn Screening: Measures immunoreactive trypsinogen (IRT) levels; elevated levels prompt further testing.
--> Sweat Chloride Test: Gold standard for diagnosis; chloride levels >60 mmol/L are diagnostic.
--> Genetic Testing: Identifies mutations in the CFTR gene for diagnosis and treatment guidance.
--> Sputum Culture: Regular analysis to identify pathogens and guide antibiotic therapy.
--> Pulmonary Function Tests (PFTs): Assess lung function and monitor disease progression.
--> Chest Radiography and CT: Evaluate lung disease severity and identify complications.
--> Blood Tests: Screen for cystic fibrosis-related diabetes via oral glucose tolerance tests.
--> Bone Density Assessment: DXA scans evaluate bone mineral density and identify osteopenia or osteoporosis.
76
What is interstitial lung disease (ILD)?
ILD includes conditions causing inflammation and fibrosis of lung parenchyma, leading to the replacement of functional lung tissue with non-functional scar tissue.
Key conditions include:
Idiopathic pulmonary fibrosis (IPF)
Secondary pulmonary fibrosis
Hypersensitivity pneumonitis
Cryptogenic organizing pneumonia
Asbestosis
77
What are the key presenting features of interstitial lung disease?
Shortness of breath on exertion
Dry cough
Fatigue
In IPF, expect:
Bibasal fine end-inspiratory crackles
Finger clubbing
78
What are the diagnostic methods for interstitial lung disease?
High-Resolution CT Scan (HRCT): Shows “ground glass” appearance.
Spirometry: May be normal or show a restrictive pattern (FEV1 and FVC reduced, FEV1 >70%).
Other Investigations: If unsure, consider lung biopsy or bronchoalveolar lavage (BAL).
79
What is the general management strategy for interstitial lung disease?
Management is primarily supportive due to poor prognosis:
Remove or treat underlying causes
Home oxygen therapy for hypoxia
Smoking cessation
Physiotherapy and pulmonary rehabilitation
Vaccinations (e.g., pneumococcal, flu)
Advanced care planning and palliative care
Lung transplant (evaluate risks and benefits)
80
What is idiopathic pulmonary fibrosis (IPF) and its management?
IPF features progressive pulmonary fibrosis with no known cause, presenting insidiously over 3+ months. It primarily affects adults over 50 years old, with a poor prognosis (2-5 years life expectancy).
Medications:
Pirfenidone: Reduces fibrosis and inflammation.
Nintedanib: Inhibits tyrosine kinase to reduce fibrosis.
81
What are the causes of secondary pulmonary fibrosis?
Secondary pulmonary fibrosis can be caused by:
Medications (MANC): Methotrexate, Amiodarone, Nitrofurantoin, Cyclophosphamide.
Conditions: Alpha-1 antitrypsin deficiency, rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, sarcoidosis.
82
What is hypersensitivity pneumonitis and its management?
Hypersensitivity pneumonitis (extrinsic allergic alveolitis) is an immune response to inhaled allergens, leading to lung inflammation.
Management:
Remove allergen
Provide oxygen if needed
Administer steroids
Examples of Causes: Bird-fancier’s lung, farmer’s lung, mushroom worker’s lung, malt worker’s lung.
83
What is cryptogenic organizing pneumonia (COP) and how is it treated?
COP involves focal lung inflammation, which can be idiopathic or triggered by infections, inflammatory disorders, medications, or allergens.
Presentation: Symptoms resemble infectious pneumonia.
Diagnosis: Lung biopsy is definitive
Treatment involves systemic corticosteroids.
84
What is pneumonia?
Pneumonia is an infection of the lung tissue, causing inflammation in the alveolar space, often seen as consolidation on a chest X-ray.
85
How is pneumonia classified based on acquisition?
Community-acquired pneumonia (CAP): Develops in the community.
Hospital-acquired pneumonia (HAP): Develops after more than 48 hours in a hospital.
Ventilator-acquired pneumonia (VAP): Develops in intubated patients in the intensive care unit.
Aspiration pneumonia: Develops due to aspiration of food or fluids, associated with anaerobic bacteria.
86
What are the presenting symptoms of pneumonia?
Cough
Sputum production
Shortness of breath
Fever
General malaise
Haemoptysis (coughing up blood)
Pleuritic chest pain (sharp pain worsened by inspiration)
Delirium (acute confusion)
87
What are the characteristic chest signs of pneumonia?
Bronchial breath sounds (harsh inspiratory and expiratory sounds)
Focal coarse crackles
Dullness to percussion
88
What observations may indicate sepsis secondary to pneumonia?
Tachypnoea (raised respiratory rate)
Tachycardia (raised heart rate)
Hypoxia (low oxygen levels)
Hypotension (shock)
Fever
Confusion
89
What is the CRB-65 scoring system?
The CRB-65 score assesses pneumonia severity in out-of-hospital patients:
C: Confusion (new disorientation)
U: Urea > 7 mmol/L
R: Respiratory rate ≥ 30
B: Blood pressure < 90 systolic or ≤ 60 diastolic
65: Age ≥ 65 years
90
What are the CURB-65 scoring system categories?
The CURB-65 score predicts mortality in hospitalized patients:
Score 0/1: Low risk (< 3% mortality) – consider home treatment.
Score 2: Intermediate risk (3-15% mortality) – consider hospital admission.
Score ≥ 3: High risk (> 15% mortality) – consider intensive care.
91
What are the top causes of typical bacterial pneumonia?
Streptococcus pneumoniae
Haemophilus influenzae
92
What are the causes of atypical pneumonia?
Atypical pneumonia is caused by organisms that are difficult to culture. Common causes include:
Legionella pneumophila (Legionnaires’ disease)
Mycoplasma pneumoniae
Chlamydophila pneumoniae
Coxiella burnetii (Q fever)
Chlamydia psittaci
93
What is Pneumocystis jirovecii pneumonia (PCP), what are its symptoms and treatment?
PCP is a fungal pneumonia occurring in immunocompromised patients, especially those with poorly controlled HIV. Symptoms include a dry cough, shortness of breath on exertion, and night sweats. Treatment involves co-trimoxazole (Septrin).
94
What investigations are performed for pneumonia?
For CRB 0 or 1 pneumonia: Typically no investigations needed.
For hospitalized patients: Chest X-ray, full blood count, renal profile, C-reactive protein.
For moderate/severe infections: Sputum cultures, blood cultures, and urinary antigen tests for pneumococcus and Legionella.
95
What is the antibiotic treatment for pneumonia?
Treatment varies based on severity:
Mild CAP: 5 days of oral antibiotics (e.g., Amoxicillin, Doxycycline, Clarithromycin).
Moderate/severe pneumonia: Initial intravenous antibiotics, transitioning to oral as improvement occurs, plus respiratory support as needed.
96
What are the complications of pneumonia?
Sepsis
Acute respiratory distress syndrome (ARDS)
Pleural effusion
Empyema
Lung abscess
Death
97
What are the key risk factors for lung cancer and their associated risk multipliers?
Smoking: increases risk by a factor of 10
Asbestos: increases risk by a factor of 5
Other factors: Arsenic, radon, nickel, chromate, aromatic hydrocarbons, cryptogenic fibrosing alveolitis
Not related: Coal dust
98
What are the main histological subtypes of lung cancer?
Lung cancers are classified as small cell lung cancer (SCLC) and non-small cell lung cancer (NSCLC), with NSCLC being the most common, accounting for 80% of all cases.
99
What are the main types of non-small cell lung cancer and their prevalence?
Squamous cell carcinoma (25% of cases)
Adenocarcinoma (40% of cases)
Large cell carcinoma (10% of cases)
100
What is the key characteristic of small cell lung carcinoma?
Small cell lung carcinoma is characterized by neuroendocrine differentiation, is strongly associated with smoking, disseminates early, and is usually chemosensitive but rarely results in long-lasting remissions.
101
What are common symptoms directly related to the primary tumor in lung cancer?
Persistent cough (dry or productive)
Haemoptysis (coughing up blood)
Dyspnoea (shortness of breath)
Chest pain (dull or sharp if pleural involvement)
Wheezing or stridor (airway obstruction)
Recurrent chest infections
Anorexia and weight loss
102
What symptoms may indicate regional spread or distant metastasis of lung cancer?
Regional Spread Symptoms:
Hoarseness (recurrent laryngeal nerve involvement)
Dysphagia (oesophageal compression)
Superior vena cava obstruction (facial swelling, distended neck veins)
Pancoast tumour symptoms (shoulder pain, Horner's syndrome)
Distant Metastasis Symptoms:
Bone pain (especially in the spine, pelvis, long bones)
Neurological symptoms (headaches, seizures)
Abdominal pain or jaundice (liver metastases)
Adrenal insufficiency (adrenal metastasis)
103
What are some paraneoplastic syndromes associated with lung cancer?
Hypercalcaemia (due to PTHrP)
Cushing's syndrome (ectopic ACTH production)
SIADH (hyponatraemia)
Lambert-Eaton myasthenic syndrome (LEMS) - NM disorder - antibodies against voltage-gated channel proteins in the presynaptic nerve terminals
Clubbing (finger deformity in chronic diseases)
104
What are the criteria for urgent referral or chest X-ray for suspected lung cancer according to NICE guidelines?
Urgent Referral (2-week appointment):
Chest X-ray findings suggestive of lung cancer.
Age 40+ with unexplained haemoptysis.
Urgent Chest X-ray (within 2 weeks) for those aged 40+ with 2+ unexplained symptoms or smokers with 1+ unexplained symptoms:
Cough
Fatigue
Shortness of breath
Chest pain
Weight loss
Appetite loss
Consider Urgent Chest X-ray (within 2 weeks) for age 40+ with any of the following:
Persistent/recurrent chest infection
Finger clubbing
Supraclavicular or persistent cervical lymphadenopathy
Chest signs consistent with lung cancer
Thrombocytosis
105
What are the key investigations for suspected lung cancer?
Chest X-ray: Often the first investigation; 10% of lung cancer cases may have a normal result.
CT scan: The investigation of choice for suspected lung cancer.
Bronchoscopy: Allows for biopsy to obtain a histological diagnosis, sometimes aided by endobronchial ultrasound.
PET scan: Used in non-small cell lung cancer to establish eligibility for curative treatment, improving sensitivity for local and distant metastasis using 18-fluorodeoxyglucose.
106
What are the key aspects of lung cancer diagnosis and management?
Staging: ~75% of lung cancer patients present with stage III/IV disease, impacting treatment options.
Histological Subtypes:
Non-small cell lung cancer (NSCLC): 85% of cases, includes squamous cell carcinoma, adenocarcinoma, and large cell carcinoma.
Small cell lung cancer (SCLC): 15% of cases, more aggressive with a worse prognosis.
Management Principles:
Multi-disciplinary team (MDT) discussion for treatment planning.
Smoking Cessation: Essential for improving treatment success and survival.
NSCLC Management:
Surgery: Lobectomy for stages I/II in medically fit patients.
Radiotherapy: First-line for inoperable stage I-III.
Chemotherapy: For stages III/IV; first-line includes a third-generation agent + platinum agent.
Combination Therapy: Adjuvant chemotherapy/radiotherapy based on surgical outcomes.
SCLC Management:
Surgery: Rarely used; primarily for very early stage.
Limited-stage: 4-6 cycles of cisplatin-based chemotherapy + radiotherapy if responsive.
Extensive-stage: Platinum-based therapy with reassessment after cycles; radiotherapy if responsive.
Relapse: Second-line therapy may include further chemotherapy or palliative radiotherapy.
107
Which cancers are commonly associated with lung metastases, and what are 'cannonball metastases'?
Lung metastases can arise from various cancers, including breast, colorectal, renal cell, bladder, and prostate cancer. 'Cannonball metastases' refer to multiple, round well-defined lung secondaries, most commonly seen with renal cell cancer, but also associated with choriocarcinoma and prostate cancer. Calcification in lung metastases is rare, typically only seen in chondrosarcoma or osteosarcoma.
108
What are the predisposing factors for obstructive sleep apnoea?
Obesity
Macroglossia (due to conditions like acromegaly, hypothyroidism, or amyloidosis)
Large tonsils
Marfan's syndrome
109
What complaints might a partner report about someone with obstructive sleep apnoea?
Excessive snoring
Periods of apnoea (temporary cessation of breathing during sleep)
110
What are the consequences of untreated obstructive sleep apnoea?
Daytime somnolence (sleepiness)
Compensated respiratory acidosis
Hypertension
111
How is sleepiness assessed in obstructive sleep apnoea?
Epworth Sleepiness Scale: A questionnaire completed by the patient and possibly their partner
Multiple Sleep Latency Test (MSLT): Measures the time it takes to fall asleep in a dark room using EEG criteria
112
What diagnostic tests are used for obstructive sleep apnoea?
Sleep studies (polysomnography): Ranging from pulse oximetry monitoring to full polysomnography that measures EEG, respiratory airflow, thoraco-abdominal movement, snoring, and pulse oximetry
113
What are the management options for obstructive sleep apnoea?
Weight loss
Continuous Positive Airway Pressure (CPAP): First-line for moderate or severe obstructive sleep apnoea
Intra-oral devices (e.g., mandibular advancement): Used if CPAP is not tolerated or for mild cases without daytime sleepiness
The DVLA should be informed if excessive daytime sleepiness is present
Limited evidence supports the use of pharmacological agents
114
What is a pulmonary embolism (PE)?
A pulmonary embolism (PE) is a blood clot (thrombus) in the pulmonary arteries, usually originating from a deep vein thrombosis (DVT) in the leg. It blocks blood flow to the lungs and strains the right side of the heart. PE and DVT are collectively known as venous thromboembolism (VTE).
115
What are the key risk factors for developing a pulmonary embolism (PE)?
Immobility
Recent surgery
Long-haul travel
Pregnancy
Hormone therapy (e.g., contraceptive pill, HRT)
Malignancy
Polycythaemia
Systemic lupus erythematosus (SLE)
Thrombophilia
116
What is VTE prophylaxis in hospitalised patients?
Low molecular weight heparin (e.g., enoxaparin) unless contraindicated
Anti-embolic compression stockings unless contraindicated (e.g., peripheral arterial disease)
117
What are the main clinical features of pulmonary embolism (PE)?
Shortness of breath
Cough
Haemoptysis (coughing up blood)
Pleuritic chest pain (sharp pain on inspiration)
Hypoxia
Tachycardia
Raised respiratory rate
Low-grade fever
Hypotension (in haemodynamic instability)
118
What diagnostic tests are used to rule out pulmonary embolism (PE)?
Wells Score: Predicts the probability of PE
D-dimer: Sensitive but not specific for VTE, used when Wells Score suggests low probability of PE
CT pulmonary angiogram (CTPA): First-line imaging for PE
Ventilation-perfusion (V/Q) scan: Used when CTPA is contraindicated (e.g., renal impairment)
119
What is the management of a suspected or confirmed pulmonary embolism (PE)?
Anticoagulation (first-line: apixaban or rivaroxaban)
Oxygen if required
Analgesia if required
Thrombolysis in massive PE with haemodynamic compromise
Monitor for deterioration
120
What are the long-term anticoagulation options for VTE?
Direct-acting oral anticoagulants (DOACs): Apixaban, rivaroxaban, edoxaban, dabigatran
Warfarin: Target INR of 2-3
Low molecular weight heparin (LMWH): First-line in pregnancy
121
How long should anticoagulation be continued after a PE?
3 months for reversible causes
Beyond 3 months for unprovoked PE, recurrent VTE, or irreversible causes
3-6 months in active cancer
122
What is the 2-level PE Wells score used for?
The 2-level PE Wells score is used to assess the clinical probability of pulmonary embolism (PE).
123
What clinical features are included in the 2-level PE Wells score, and how many points are assigned to each?
Clinical signs/symptoms of DVT (e.g., leg swelling, pain with palpation): 3 points
An alternative diagnosis less likely than PE: 3 points
Heart rate > 100 bpm: 1.5 points
Immobilisation for >3 days or surgery in the past 4 weeks: 1.5 points
Previous DVT/PE: 1.5 points
Haemoptysis: 1 point
Malignancy (active or treated in the last 6 months, or palliative): 1 point
124
How are the results of the 2-level PE Wells score interpreted?
PE likely: More than 4 points
PE unlikely: 4 points or less
125
What is the next step if the 2-level PE Wells score suggests PE is 'likely' (more than 4 points)?
Arrange an immediate CT pulmonary angiogram (CTPA)
If there is a delay in performing the CTPA, start interim therapeutic anticoagulation (usually with a DOAC such as apixaban or rivaroxaban)
If the CTPA is positive, a PE is diagnosed
If the CTPA is negative, consider a proximal leg vein ultrasound if DVT is suspected
126
What is the next step if the 2-level PE Wells score suggests PE is 'unlikely' (4 points or less)?
Arrange a D-dimer test
If the D-dimer is positive, arrange an immediate CT pulmonary angiogram (CTPA) and start interim anticoagulation if there is a delay
If the D-dimer is negative, stop anticoagulation and consider an alternative diagnosis
127
What are the ECG changes associated with PE?
RBBB and right axis deviation
Large S wave in Lead 1, Large Q wave seen in Lead 3, Inverted T wave seen in lead 3 - S1Q3T3
Sinus tachycaria
128
What is pulmonary hypertension, and how is it defined?
Pulmonary hypertension is increased resistance and pressure in the pulmonary arteries, causing strain on the right heart. It is defined as a mean pulmonary arterial pressure of more than 20 mmHg.
129
What are the five groups of causes for pulmonary hypertension?
Idiopathic pulmonary hypertension or connective tissue disease (e.g., systemic lupus erythematous)
Left heart failure (e.g., myocardial infarction or systemic hypertension)
Chronic lung disease (e.g., COPD or pulmonary fibrosis)
Pulmonary vascular disease (e.g., pulmonary embolism)
Miscellaneous causes (e.g., sarcoidosis, glycogen storage disease)
130
What are the key signs and symptoms of pulmonary hypertension?
Shortness of breath
Syncope
Tachycardia
Raised jugular venous pressure (JVP)
Hepatomegaly
Peripheral oedema
131
What are the investigations for pulmonary hypertension?
ECG: P pulmonale (peaked P waves) , right ventricular hypertrophy, right axis deviation, right bundle branch block
Chest x-ray: Dilated pulmonary arteries, right ventricular hypertrophy
Raised NT‑proBNP blood test result indicates right ventricular failure
Echocardiogram can be used to estimate the pulmonary artery pressure
132
What are the treatment options for idiopathic pulmonary hypertension?
Calcium channel blockers
Intravenous prostaglandins (e.g., epoprostenol)
Endothelin receptor antagonists (e.g., macitentan)
Phosphodiesterase-5 inhibitors (e.g., sildenafil)
Secondary pulmonary hypertension is managed by treating the underlying cause, such as pulmonary embolism, COPD or systemic lupus erythematosus.
Supportive treatments (e.g., oxygen and diuretics) are used for complications such as respiratory failure, oedema and arrhythmias.
133
How is secondary pulmonary hypertension managed?
By treating the underlying cause, such as pulmonary embolism, COPD, or systemic lupus erythematosus, along with supportive treatments like oxygen and diuretics.
134
What is respiratory failure, and how is it defined?
Respiratory failure occurs when there is a failure of gas exchange and/or ventilation, leading to abnormal PaO2 (oxygen) and/or PaCO2 (carbon dioxide) on arterial blood gas.
135
What are the two broad categories of respiratory failure?
Type 1 respiratory failure: Hypoxaemia (PaO2 < 8 kPa / 60 mmHg) with normocapnia (PaCO2 < 6 kPa / 45 mmHg).
Type 2 respiratory failure: Hypoxaemia (PaO2 < 8 kPa / 60 mmHg) with hypercapnia (PaCO2 > 6 kPa / 45 mmHg).
136
What characterizes type 1 respiratory failure?
Type 1 respiratory failure involves hypoxaemia (PaO2 < 8 kPa) with normocapnia (PaCO2 < 6 kPa).
137
What is the main underlying cause of type 1 respiratory failure?
Ventilation/perfusion (V/Q) mismatch, where air flow (ventilation) does not match blood flow (perfusion) in the lungs.
138
What causes type 1 respiratory failure?
Reduced ventilation with normal perfusion (e.g., pneumonia, pulmonary oedema, bronchoconstriction)
Reduced perfusion with normal ventilation (e.g., pulmonary embolism)
139
What characterizes type 2 respiratory failure?
Type 2 respiratory failure involves hypoxaemia (PaO2 < 8 kPa) with hypercapnia (PaCO2 > 6 kPa).
140
What is the main underlying cause of type 2 respiratory failure?
Alveolar hypoventilation, leading to inadequate oxygenation and elimination of CO2 from the blood.
141
What causes type 2 respiratory failure?
Increased airway resistance (e.g., COPD)
Reduced lung/chest wall compliance (e.g., pneumonia, rib fractures, obesity)
Reduced respiratory muscle strength (e.g., Guillain-Barré, motor neurone disease)
Reduced respiratory drive (e.g., opioids, sedatives)
142
What is sarcoidosis?
Sarcoidosis is a chronic granulomatous disorder characterized by the formation of inflammatory granulomas. The cause is unknown.
143
What are the typical demographics for sarcoidosis?
Sarcoidosis is more common in people aged 20-39 or around 60, more frequent in women and those of Black ethnic origin.
144
What is a typical patient presentation in exams for sarcoidosis?
A 20-40 year old Black female with a dry cough, shortness of breath, and erythema nodosum on the shins.
145
What are two skin manifestations of sarcoidosis?
Erythema nodosum: raised, red, tender nodules on the shins.
Lupus pernio: raised purple lesions on the cheeks and nose, specific to sarcoidosis.
146
Which organs can sarcoidosis affect?
It can affect almost any organ, with lungs (over 90% of patients) being the most commonly involved.
147
What are common lung manifestations of sarcoidosis?
Mediastinal lymphadenopathy
Pulmonary fibrosis
Pulmonary nodules
148
What are some systemic symptoms of sarcoidosis?
Fever
Fatigue
Weight loss
149
What are some liver manifestations of sarcoidosis?
Liver nodules
Cirrhosis
Cholestasis
150
How can sarcoidosis affect the eyes?
Uveitis
Conjunctivitis
Optic neuritis
151
How can sarcoidosis affect the heart?
Bundle branch block
Heart block
Myocardial involvement
152
What are the kidney manifestations of sarcoidosis?
Kidney stones (due to hypercalcaemia)
Nephrocalcinosis
Interstitial nephritis
153
How does sarcoidosis affect the central nervous system?
Nodules
Pituitary involvement (diabetes insipidus)
Encephalopathy
154
What are two peripheral nervous system manifestations of sarcoidosis?
Facial nerve palsy
Mononeuritis multiplex
155
How can sarcoidosis affect the bones?
Arthralgia
Arthritis
Myopathy
156
What is Lofgren’s syndrome?
Erythema nodosum
Bilateral hilar lymphadenopathy
Polyarthralgia (joint pain in multiple joints)
157
What blood test results are indicative of sarcoidosis?
Raised ACE (angiotensin-converting enzyme)
Raised calcium (hypercalcaemia)
158
What are key imaging investigations in sarcoidosis?
Chest X-ray: shows hilar lymphadenopathy
High-resolution CT: shows lymphadenopathy and pulmonary nodules
MRI: shows CNS involvement
PET scan: shows active inflammation
159
What are the characteristic histological findings in sarcoidosis?
Non-caseating granulomas with epithelioid cells, usually confirmed via bronchoscopy with biopsy.
160
What is the first-line treatment for sarcoidosis?
Oral steroids (6-24 months) are first-line. Bisphosphonates are used to protect against osteoporosis during long-term steroid use.
161
What is a second-line treatment for sarcoidosis?
Methotrexate is a second-line option.
162
What is the management approach for sarcoidosis?
Conservative Management:
Considered for patients with no or mild symptoms.
Medical Management:
First-line: Oral steroids (6-24 months)
Bisphosphonates used to protect against osteoporosis during long-term steroid use.
Second-line: Methotrexate
Severe Pulmonary Disease:
Lung transplant (rarely required)
163
What are the key manifestations of sarcoidosis, grouped by organ systems?
Pulmonary:
Mediastinal lymphadenopathy
Pulmonary fibrosis
Pulmonary nodules
Systemic:
Fever
Fatigue
Weight loss
Skin:
Erythema nodosum
Lupus pernio
Liver:
Liver nodules
Cirrhosis
Cholestasis
Eyes:
Uveitis
Conjunctivitis
Optic neuritis
Heart:
Bundle branch block
Heart block
Myocardial muscle involvement
Kidneys:
Kidney stones (hypercalcaemia)
Nephrocalcinosis
Interstitial nephritis
Central Nervous System (CNS):
Nodules
Pituitary involvement (diabetes insipidus)
Encephalopathy
Peripheral Nervous System (PNS):
Facial nerve palsy
Mononeuritis multiplex
Musculoskeletal:
Arthralgia
Arthritis
Myopathy
Lofgren’s Syndrome:
Erythema nodosum
Bilateral hilar lymphadenopathy
Polyarthralgia
164
What is the causative agent of tuberculosis (TB), and how does it spread?
Tuberculosis is caused by Mycobacterium tuberculosis, an aerobic, acid-fast bacillus, primarily spread via droplet infection.
165
What are the risk factors for tuberculosis (TB)?
Immunosuppression (e.g., HIV, immunosuppressant drugs, TNFα inhibitors)
Diabetes mellitus, end-stage renal disease
Previous lung disease (e.g., silicosis)
Smoking, drug abuse, alcoholism
Malnutrition, poverty
Certain living conditions (e.g., prisons, homeless shelters)
Occupational risk (e.g., healthcare workers)
166
What are the special staining characteristics of Mycobacterium tuberculosis?
--> M. tuberculosis has a waxy coating that makes gram staining ineffective. It is an acid-fast bacillus, meaning it is resistant to acids used in staining. It is stained with the --> Zeihl-Neelsen stain, turning bright red against a blue background.
167
What are the four possible outcomes after Mycobacterium tuberculosis enters the body?
--> Immediate clearance of the bacteria
--> Primary active tuberculosis
--> Latent tuberculosis
--> Secondary tuberculosis (reactivation of latent TB)
168
What is latent tuberculosis, and how does it differ from secondary tuberculosis?
Latent tuberculosis occurs when the immune system encapsulates the bacteria, stopping disease progression without symptoms. Secondary tuberculosis is the reactivation of latent TB, usually due to immunosuppression, leading to active infection.
169
What are the common sites for extrapulmonary tuberculosis?
Lymph nodes
Pleura
Central nervous system
Pericardium
Gastrointestinal system
Genitourinary system
Bones and joints
Skin (cutaneous TB)
170
What is miliary tuberculosis, and what is a characteristic radiological finding?
Miliary tuberculosis refers to disseminated TB infection throughout the body. On chest x-ray, it appears as small, uniformly distributed nodules resembling millet seeds.
171
What are the clinical features of tuberculosis (TB)?
--> Latent TB: Asymptomatic and non-contagious.
--> Active TB: Constitutional: Gradual fever, night sweats, weight loss, anorexia, malaise.
--> Pulmonary TB: Cough (>2-3 weeks),
initially dry then productive,
haemoptysis, dyspnoea, chest pain.
--> Extrapulmonary TB: Affects pleura,
bones, lymphatic system, CNS, liver,
urogenital and GI tracts, skin.
Symptoms vary based on organ
involvement (e.g., lymphadenopathy,
pleuritic chest pain, skeletal pain,
(urinary symptoms).
172
What are the key investigations for tuberculosis (TB)?
Latent TB:
--> Tuberculin Skin Test (TST): Measures induration 48–72 hours after intradermal injection.
--> Interferon-Gamma Release Assay (IGRA): Measures interferon-γ levels in blood.
Active TB:
--> Chest X-ray: Hilar lymphadenopathy, pleural effusion, upper lobe cavitary lesions (reactivation).
--> Sputum Microscopy: Ziehl-Neelsen stain for acid-fast bacilli.
--> Culture (Gold standard): Slow but highly specific; culture techniques using liquid media can give faster results.
--> Nucleic Acid Amplification Test (NAAT): Detects TB DNA and drug resistance genes
173
What is the management for tuberculosis (TB)?
--> Latent TB:
Isoniazid and rifampicin for 3 months
Isoniazid for 6 months
--> Active TB:
- Intensive phase: Isoniazid, rifampin, pyrazinamide, and ethambutol for 2 months.
- Continuation phase: Isoniazid and rifampin for 4 months.
- Monitor monthly sputum cultures until two consecutive negative results.
--> Multidrug-Resistant TB (MDR-TB): Regimen with second-line drugs for 9–18 months.
Common drugs: Levofloxacin, bedaquiline, linezolid.
174
What side effects are associated with the RIPE drugs for tuberculosis treatment?
Rifampicin: Red/orange secretions, CYP450 inducer (affects drugs like the contraceptive pill)
Isoniazid: Peripheral neuropathy (prevented by pyridoxine), hepatotoxicity
Pyrazinamide: Hyperuricaemia, gout, kidney stones, hepatotoxicity
Ethambutol: Colour blindness, reduced visual acuity
175
What is the pathophysiology of Goodpasture's syndrome?
Goodpasture's syndrome is an autoimmune disease where antibodies attack the alpha-3 subunit of type IV collagen in the basement membrane of the lungs and kidneys. This leads to small vessel vasculitis, causing pulmonary hemorrhage and renal failure.
176
What happens if Goodpasture's syndrome is left untreated?
If left untreated, Goodpasture's syndrome causes permanent damage to the lungs and kidneys and can lead to death. With treatment, the five-year survival rate exceeds 80%.
177
What factors contribute to the development of Goodpasture's syndrome?
Genetic Predisposition: Certain HLA subtypes, especially HLA-DRB1, increase susceptibility.
Environmental Triggers: Exposure to hydrocarbons, tobacco smoke, or viral respiratory infections may trigger the disease.
Immunological Factors: Circulating anti-GBM antibodies target type IV collagen in the basement membranes of the lungs and kidneys, causing damage.
178
What initiates the autoimmune response in Goodpasture's syndrome?
Environmental triggers or other factors in susceptible individuals lead to the production of anti-GBM antibodies by B-cells.
179
Where do anti-GBM antibodies deposit in Goodpasture's syndrome?
Anti-GBM antibodies deposit on the glomerular and alveolar basement membranes, which are rich in type IV collagen.
180
What is the role of immune complex formation and complement activation in Goodpasture's syndrome?
Immune complex formation leads to complement activation, which releases chemotactic factors and cytokines, enhancing inflammation and tissue damage.
181
How do inflammatory cells cause tissue damage in Goodpasture's syndrome?
Neutrophils and macrophages infiltrate the tissue, releasing reactive oxygen species (ROS) and proteolytic enzymes, resulting in crescentic glomerulonephritis in the kidneys and alveolar hemorrhage in the lungs.
182
What are the long-term consequences of untreated Goodpasture's syndrome?
Ongoing inflammation leads to irreversible damage, causing renal scarring, fibrosis, and chronic lung injury.
183
What are the clinical features, symptoms, signs, and risk factors of Goodpasture's syndrome?
Clinical Presentation: Abrupt onset of:
--> Haemoptysis
--> Cough
--> Shortness of breath
--> Peripheral oedema
--> Dark urine
--> Oliguria
Symptoms:
Haemoptysis: 65%
Cough: 30%
Shortness of breath: 30%
Nausea and vomiting: 20%
Chest pain: 20%
Decreased urine output: 15%
Fatigue and malaise
Haematuria: 5%
Signs:
Lung crackles: 45%
Fever: 30%
Lower extremity oedema: 15%
Risk Factors:
Male-to-female ratio: 3:2
Bimodal age distribution:
20-30 years
60-70 years
184
What investigations are recommended for diagnosing Goodpasture's syndrome
--> Renal Biopsy
- Definitive diagnosis
- Typically shows crescentic glomerulonephritis
- Linear IgG staining on
immunofluorescence
--> Blood Tests
- Anti-GBM Antibody Titre
Useful confirmatory diagnostic test alongside renal biopsy
- Anti-Neutrophil Cytoplasmic Antibodies (ANCA)
Positive in up to 30% of patients with anti-GBM disease
- Urea & Electrolytes
High levels of urea and creatinine
- Urinalysis
Proteinuria, hematuria, and casts
--> Chest Imaging
-X-ray may show diffuse opacities
-CT Scan - Characteristically shows ground glass or consolidative opacities in a diffuse and bilateral distribution
185
What are the main differential diagnoses for Goodpasture's syndrome?
ANCA Vasculitides (e.g., granulomatosis with polyangiitis, microscopic polyangiitis, Churg-Strauss syndrome)
Similarities: Blood tests may show positive ANCA.
Differences: Renal biopsy shows no immune complex deposition.
Post-Streptococcal Glomerulonephritis
Similarities: Presents only with glomerulonephritis.
Differences: Renal biopsy shows immune complex deposition in a granular pattern, unlike Goodpasture's syndrome.
186
What is the management for Goodpasture's syndrome?
Acute Management:
Intensive plasmapheresis:
Removes pathogenic antibodies and inflammatory mediators.
4 litres per day for 10-14 days or until anti-GBM is undetectable.
Prednisone:
Dosage is tapered over 3 months.
Cyclophosphamide:
Immunosuppressive treatment.
Long-term Management:
--> Optimal duration of treatment is unknown; cessation of autoantibody formation may take 6 to 9 months.
--> Maintenance therapy may include less toxic drugs (e.g., azathioprine) and low-dose prednisolone.
--> Smoking cessation: Important to reduce glomerular and alveolar damage, which releases more auto-antigens.
187
What is the protein concentration that classifies pleural effusions as a transudate?
Transudate is classified as having a protein concentration of <30 g/L.
188
What are common causes of transudate pleural effusions?
Heart failure (most common cause)
Hypoalbuminaemia (liver disease, nephrotic syndrome, malabsorption)
Hypothyroidism
Meigs' syndrome
189
What is the protein concentration that classifies pleural effusions as an exudate?
Exudate is classified as having a protein concentration of >30 g/L.
190
What are common causes of exudate pleural effusions?
Infection: pneumonia (most common cause), TB, subphrenic abscess
Connective tissue diseases: RA, SLE
Neoplasia: lung cancer, mesothelioma, metastases
Pancreatitis
Pulmonary embolism
Dressler's syndrome
Yellow nail syndrome
191
What are Light's criteria for distinguishing an exudative pleural effusion?
1. Pleural fluid protein / serum protein > 0.5
2. Pleural fluid LDH / serum LDH > 0.6
3. Pleural fluid LDH > 2/3 of the normal upper limit of serum LDH.
192
What are common clinical features of pleural effusions?
Dyspnoea, non-productive cough, chest pain.
193
What classic examination findings may indicate a pleural effusion?
Dullness to percussion, reduced breath sounds, reduced chest expansion.
194
What imaging and procedures are recommended by the British Thoracic Society for investigating pleural effusions?
Imaging:
PA chest x-rays for all patients
Ultrasound to increase the likelihood of successful aspiration
Contrast CT to investigate the underlying cause, especially for exudative effusions
Pleural Aspiration:
Use ultrasound to reduce complications
Use a 21G needle and 50ml syringe
Send fluid for pH, protein, LDH, cytology, and microbiology
195
What are some characteristic pleural fluid findings and their associations?
Low Glucose: Rheumatoid arthritis, tuberculosis
Raised Amylase: Pancreatitis, oesophageal perforation
Heavy Blood Staining: Mesothelioma, pulmonary embolism, tuberculosis
196
What is the management for patients with pleural effusion associated with sepsis or pneumonia?
Perform diagnostic pleural fluid sampling
If the fluid is purulent or turbid, place a chest tube for drainage
If the fluid is clear but the pH < 7.2, place a chest tube
197
What are the management options for recurrent pleural effusion?
Recurrent aspiration.
Pleurodesis.
Indwelling pleural catheter.
Drug management to alleviate symptoms (e.g., opioids for dyspnoea).
198
What is empyema, and how is it diagnosed and treated?
Empyema is an infected pleural effusion. It should be suspected in patients with improving pneumonia but a new or ongoing fever. Diagnosis involves pleural aspiration, which shows pus, low pH, low glucose, and high LDH. Treatment includes placement of a chest drain and antibiotics.
199
What are the risk factors for pneumothorax?
Primary Spontaneous Pneumothorax:
More prevalent in tall, lean males
Often linked to subpleural bleb rupture
Pre-existing Lung Disease:
COPD (Chronic Obstructive Pulmonary Disease)
Asthma
Cystic fibrosis
Lung cancer
Pneumocystis pneumonia
Connective Tissue Disease:
Marfan's syndrome
Rheumatoid arthritis
Ventilation:
Including non-invasive ventilation
Catamenial Pneumothorax:
Accounts for 3-6% of spontaneous pneumothoraces in menstruating women
Thought to be caused by endometriosis within the thorax
200
What is the underlying mechanism of pneumothorax, and what can occur as a result?
Pressure Disruption:
Normal pleural space has slightly negative pressure, keeping lungs inflated.
Breach in lung or chest wall allows air into pleural space, disrupting pressure gradient.
Accumulation of air increases intrapleural pressure, leading to lung collapse due to elastic recoil.
Tension Pneumothorax:
If air acts as a one-way valve, tension pneumothorax develops.
Accumulated air compresses mediastinum, impairing venous return to the heart.
Results in decreased cardiac output and potential circulatory collapse.
Inflammatory Reactions:
Presence of air may cause pleural pain or pleurisy.
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What are the classifications of pneumothoraces?
Spontaneous Pneumothorax:
Primary Spontaneous Pneumothorax (PSP):
Occurs without underlying lung disease.
Common in tall, thin, young individuals.
Associated with rupture of subpleural blebs or bullae.
Secondary Spontaneous Pneumothorax (SSP):
Occurs in patients with pre-existing lung disease (e.g., COPD, asthma, cystic fibrosis, lung cancer, Pneumocystis pneumonia).
Connective tissue diseases (e.g., Marfan's syndrome) are also risk factors.
Traumatic Pneumothorax:
Results from penetrating or blunt chest trauma, leading to lung injury and air accumulation in the pleural space.
Iatrogenic Pneumothorax:
Occurs as a complication of medical procedures (e.g., thoracentesis, central venous catheter placement, lung biopsy).
Tension Pneumothorax:
A severe form resulting in mediastinal displacement, causing severe respiratory distress and hemodynamic collapse.
Catamenial Pneumothorax:
Accounts for 3-6% of spontaneous pneumothoraces in menstruating women, thought to be caused by thoracic endometriosis.
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What are the causes of pneumothorax?
Spontaneous: Primary and secondary (due to underlying lung disease).
Trauma: Penetrating or blunt chest trauma.
Iatrogenic: Due to lung biopsy, mechanical ventilation, or central line insertion.
Lung Pathologies: Such as infection, asthma, or COPD.
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What are the investigations for pneumothorax?
Erect Chest X-ray: The investigation of choice for a simple pneumothorax, showing an area without lung markings.
CT Thorax: Detects small pneumothoraces not visible on chest X-ray and accurately assesses size.
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What are the British Thoracic Society (BTS) guidelines for the management of primary and secondary spontaneous pneumothorax, and what do these terms mean?
Definitions:
Primary Pneumothorax: Occurs in patients without underlying lung disease.
Secondary Pneumothorax: Occurs in patients with underlying lung disease.
Guidelines:
Primary Pneumothorax:
Rim of air < 2 cm & not short of breath: consider discharge.
Otherwise, attempt aspiration. If it fails (rim > 2 cm or still short of breath), insert a chest drain.
Secondary Pneumothorax:
If > 50 years old with rim > 2 cm or short of breath: insert a chest drain.
If rim is 1-2 cm: attempt aspiration. If it fails (rim > 1 cm), insert a chest drain. All patients should be admitted for at least 24 hours.
If rim < 1 cm: give oxygen and admit for 24 hours.
Iatrogenic Pneumothorax:
Majority resolve with observation; if treatment needed, use aspiration. Ventilated patients may require chest drains.
Persistent/Recurrent Pneumothorax:
Consider VATS for mechanical/chemical pleurodesis +/- bullectomy for persistent air leak or recurrent episodes.
Discharge Advice:
Smoking: Avoid to reduce recurrence risk.
Fitness to Fly: Travel is contraindicated until 2 weeks after drainage if no residual air; revised from 6 weeks to 1 week post check x-ray.
Scuba Diving: Avoid unless bilateral surgical pleurectomy and normal lung function
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What is tension pneumothorax?
Caused by trauma to the chest wall creating a one-way valve that lets air in but not out of the pleural space, leading to increased pressure in the thorax and possible cardiorespiratory arrest.
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What are the signs of tension pneumothorax?
Tracheal deviation away from the side of the pneumothorax.
Reduced air entry on the affected side.
Increased resonance to percussion on the affected side.
Tachycardia and hypotension.
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What is the management for tension pneumothorax?
Insert a large bore cannula into the second intercostal space in the midclavicular line. Alternatively, use the fourth or fifth intercostal space, anterior to the midaxillary line. A chest drain should follow.
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Where should a chest drain be inserted?
Inserted in the "triangle of safety," formed by:
The 5th intercostal space (or inferior nipple line).
The midaxillary line (lateral edge of the latissimus dorsi).
The anterior axillary line (lateral edge of the pectoralis major).
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What should you do after inserting a chest drain?
Obtain a chest X-ray to check the positioning of the drain.
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What indicates successful treatment with a chest drain?
Air bubbling through the fluid in the drain bottle, swinging of the water with respiration, and re-inflation of the lung on repeat chest X-ray.
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What is Granulomatosis with Polyangiitis (GPA)?
GPA is an ANCA-associated systemic vasculitis that typically affects small and medium-sized blood vessels.
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What are the other two ANCA-associated systemic vasculitides?
Eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome) and microscopic polyangiitis.
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What is the main pathological effect of GPA on blood vessels?
GPA causes inflammation and necrosis of blood vessels, leading to stenosis, occlusion, or aneurysm formation.
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What is the classic triad of organ involvement in GPA?
The classic triad involves upper and lower respiratory tract symptoms and glomerulonephritis.
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What is the suspected cause of GPA?
GPA is thought to have an autoimmune aetiology.
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What type of immune-mediated disease is Granulomatosis with Polyangiitis (GPA)?
GPA is an immune-mediated disease involving a complex immune response to endothelial damage and inflammation.
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Which antibodies are associated with GPA and other vasculitides?
GPA is associated with anti-neutrophil cytoplasmic antibodies (ANCA), along with microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA).
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What are the two types of ANCA?
The two types of ANCA are cytoplasmic ANCA (cANCA) and perinuclear ANCA (pANCA).
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What is the clinical presentation of Granulomatosis with Polyangiitis (GPA)?
--> Prodrome: Fever, malaise, arthralgias, weight loss (non-specific systemic symptoms).
Classic triad of organ involvement:
--> Upper respiratory tract (90%): Sinusitis, nasal crusting, epistaxis, nasal septal perforation, saddle nose deformity, subglottic stenosis (hoarseness/stridor), otitis media, hearing loss.
--> Lower respiratory tract (78%): Dyspnoea, cough, pleuritis, haemoptysis, pulmonary infiltrates or nodules on imaging.
--> Glomerulonephritis (75%): Microscopic haematuria, urinary sediment, pauci-immune rapidly progressive glomerulonephritis (RPGN), leading to chronic kidney disease.
Other common organ manifestations:
--> Ocular (60%): Scleritis/episcleritis (eye pain, redness, tearing), orbital mass (proptosis, diplopia, visual loss).
--> Cutaneous (50%): Leukocytoclastic angiitis (lower extremity purpura, ulceration), petechiae, nodules, vesicles.
--> Neurological (15%): Mononeuritis multiplex, peripheral sensorimotor polyneuropathy, cranial neuropathy.
Less common organ involvement: Gastrointestinal, cardiac, lower genitourinary, and endocrine systems.
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What are the key investigations for Granulomatosis with Polyangiitis (GPA)?
cANCA: Positive in over 90% of cases.
pANCA: Positive in around 25% of cases.
Chest X-ray: Can show a wide variety of presentations, including cavitating lesions.
Renal biopsy: Epithelial crescents in Bowman's capsule (indicating glomerulonephritis).
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What are the key differentials for Granulomatosis with Polyangiitis (GPA)?
Eosinophilic Granulomatosis with Polyangiitis (EGPA):
Similarities: Non-specific prodrome, upper/lower respiratory tract, renal involvement, ANCA positive.
Differences: Asthma (90%), cardiac/GI manifestations, eosinophilia.
Microscopic Polyangiitis (MPA):
Similarities: Non-specific prodrome, glomerulonephritis, ANCA positive.
Differences: Absence of upper respiratory tract involvement.
Systemic infections (e.g., sepsis, infective endocarditis):
Similarities: Constitutional symptoms, multi-organ involvement, renal/respiratory involvement, raised inflammatory markers.
Differences: Positive cultures (e.g., blood),
valvular vegetations in IE, benefit from procalcitonin.
Malignancies (e.g., pulmonary malignancy, lymphoma):
Similarities: Constitutional symptoms, respiratory tract involvement, nodules/cavities on imaging.
Differences: Absence of extrapulmonary features, biopsy positive for malignancy or lymphoma.
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What is the typical survival without treatment for GPA?
Untreated GPA has a median survival of 5 months, highlighting the need for aggressive management.
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What are the two broad components of GPA management?
Induction of remission
Maintenance of remission
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What is the first-line treatment for life- or organ-threatening GPA during induction of remission?
Methylprednisolone IV for 3-5 days (followed by oral prednisolone)
Cyclophosphamide for 3-6 months to induce remission
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What is the management approach for Granulomatosis with Polyangiitis (GPA)?
Induction of Remission:
--> For life/organ-threatening disease:
First-line: Methylprednisolone IV (3-5 days), followed by oral prednisolone + cyclophosphamide (3-6 months).
--> Second-line: Methylprednisolone IV + rituximab IV.
Plasmapheresis may be used in severe, non-responsive cases.
--> For non-life/organ-threatening disease:
First-line: Methylprednisolone IV (3-5 days), followed by oral prednisolone + methotrexate (with folic acid).
Second-line: Oral prednisolone + cyclophosphamide or rituximab.
--> Maintenance of Remission:
First-line: Prednisolone + methotrexate (with folic acid) or azathioprine.
Duration of maintenance is typically 2 years but varies.
--> Prophylaxis:
Osteoporosis and Pneumocystis jiroveci prophylaxis.
Long-term Care:
Managed with regular specialist reviews (rheumatologists, respiratory, nephrologists, etc.) and GP follow-ups.
Hospitalisation may be needed for complications (e.g., severe infections, renal/respiratory failure).
