Respiratory (COPD, bronchiectasis, pulmonary fibrosis) Flashcards
(58 cards)
1
Q
COPD
A
a progressive lung disease characterised by airflow obstruction that is not fully reversible
Umbrella term
- Chronic bronhcitis
- Emphysema
2
Q
COPD Risk Factors
A
- Cigarette smoking is associated with around 90% of cases
- Occupational exposure e.g. coal, grains, silica
- Air pollution
- Genetics e.g. alpha-1-antitrypsin deficiency
- Asthma
- Problems with lung growth and development
3
Q
presentation of COOD
A
Cough – this is usually the initial presenting complaint
- This often starts off being worse in the morning but becomes constant as COPD gets worse
- Usually productive
Shortness of breath
- This is usually with exercise initially but can progress to shortness of breath at rest
Features of hyperinflation may be seen
- Hyperinflation occurs when air is trapped in the lungs due to blockages and reduced elasticity leading to the lungs overinflating.
- Hyper-resonance on percussion
- Barrel chest
- Distant breath sounds on auscultation
Wheeze on auscultation
Coarse crackles – often seen in exacerbations
Features of cor pulmonale:
* Distended neck veins
* Lower limb oedema
* Hepatosplenomegaly
4
Q
what does COPD not cause
A
CLUBBING
5
Q
investigations for COPD
A
- Spirometry <0.70
- FBC (secondary polycythaemia)
- CXR (rule out lung cancer, may show bullae, flattened diaphragm)
Other investigations
- Alpha-1-antitrypsin (if early onset)
- ECG and echocardiography (pulmonary hypertension)
- Sputum culture
- ABG
6
Q
NICE classification of the severity of COPD
A
7
Q
General management in all COPD patients
A
- Smoking cessation – the single most important measure to reduce progression
- Annual influenza vaccination and one-off pneumococcal vaccination
- Pulmonary rehabilitation – if COPD is limiting functional ability
8
Q
medical therapy for COPD
A
First line
- short-acting beta2 agonist (SABA) or short-acting muscarinic antagonist (SAMA)
Second line determine if the patient has asthmatic/steroid-responsive features
- If asthmatic/steroid responsive features: add long-acting beta2 agonist (LABA) + inhaled corticosteroid (ICS):
- If non-asthmatic and no steroid-responsive features: LABA + long-acting muscarinic antagonist (LAMA)
- If the patient is already taking a SAMA, stop this and replace it with a SABA
Third line add whichever option was not added in the second-line step i.e. SABA + LABA + LAMA + ICS
- Consider adding theophylline
- Consider mucolytics in all patients
9
Q
COPD atntibiotic prophylaxis
A
Antibiotic prophylaxis
In some patients, specialists may consider antibiotic prophylaxis. The antibiotic of choice is azithromycin.
10
Q
Long-term oxygen therapy (LTOT) considered in COPD patients when
A
- PaO2 <7.3 kPa when stable or <8 kPa when stable + peripheral oedema
- Secondary polycythaemia
- Pulmonary hypertension
11
Q
how can COPD cause heart failure
A
Cor pulmonale:
Chronic hypoxia can lead to vasoconstriction in the pulmonary arteries (in an attempt to increase oxygenation of the blood) leading to pulmonary hypertension. This leads to more strain on the right side of the heart leading to right ventricular hypertrophy and cor pulmonale.
12
Q
single biggest factor which can reduce rate of decline
A
Smoking cessation can reduce the rate of decline
13
Q
Alpha-1 Antitrypsin Deficiency
A
Alpha-1 antitrypsin (α1AT) is a glycoprotein produced in the liver and plays a role in inhibiting the action of protease enzymes released from neutrophils, such as neutrophil elastase released in inflammation, infection, or smoking.
14
Q
pathophysiology of Alpha-1 antitrypsin (α1AT) deficiency
A
α1AT deficiency is an inherited condition in which the α1AT protein’s structure is modified.
- This can lead to excess elastin breakdown leading to the destruction of alveolar walls and COPD.
- The α1AT can accumulate in the liver, leading to liver cell destruction and cirrhosis. In exams, α1AT deficiency classically causes COPD in young people and non-smokers.
15
Q
presentation of α1AT deficiency
A
α1AT deficiency should be considered in any young patient presenting with features consistent with COPD, or any patient with severe and aggressive COPD. Features may be:
- Early presentation of COPD symptoms
- Imaging shows emphysema in the lower lobes of the lung
- Cirrhosis and hepatocellular carcinoma may be present, even in the absence of alcohol consumption
- Not every patient with α1AT deficiency will develop liver disease
16
Q
investigations for a1AT deficiency
A
Serum α1AT levels:
- All patients with COPD, non-responsive asthmatic adults/adolescents, and people with liver disease of unknown aetiology should be screened using this test
Spirometry:
- FEV1: significantly reduced
- FVC: significantly reduced
- FEV1/FVC: significantly reduced
Chest x-ray:
- Usually shows emphysematous changes – particularly in the lower lobes
Liver function tests (LFTs):
- ALT, bilirubin, and ALP may be raised
17
Q
management of a1AT def
A
- STOP smoking
- supportuve therapies such as bronchodilators and pulmonary rehab
- lung volume reduction surgery
18
Q
An acute exacerbation of chronic obstructive pulmonary disease (COPD)
A
is a sudden worsening of COPD symptoms beyond normal day-to-day variations.
19
Q
causes of COPD exacerbation
A
Bacterial causes
- Haemophilus influenzae – most common
- Streptococcus pneumoniae
- Moraxella catarrhalis
Other causes
- Pollution
- Viruses – rhinoviruses, influenza, parainfluenza etc.
- Swallowing dysfunction
- Gastro-oesophageal reflux disease (GORD)
20
Q
presentation of acute COPD exacerbation
A
- Increased shortness of breath, cough, and wheezing
- Increased sputum production which may suggest an infection
- Purulent (green/pus-filled) sputum is suggestive of a bacterial infection
- Malaise
- Signs of respiratory failure
21
Q
Signs of respiratory failure:
A
- Changes in mental status e.g. confusion
- Morning headaches and increased daytime sleepiness suggest worsening hypercapnia
- Accessory muscle use and pursed lip breathing
- Cyanosis
22
Q
investigations for acute exacerbation of COPD
A
- CXR
- ABG Used to detect chronic hypercapnia and acute respiratory acidosis- guides oxygen therapy
- FBC - infections and anaemia
- U&Es
- ECGs
- Sputum culture - bacterial infection
- Blood culture
23
Q
when pt with COPD should be referred to hospital
A
- Significantly worsened dyspnoea
- Tachypnoea
- Oxygen saturations <90%
- Pursed-lip breathing
- Use of accessory muscles
- Mental status changes e.g. confusion or impaired levels of consciousness
- New-onset cyanosis
- Worsening peripheral oedema
- Rapid onset
- Significant reduction in activities of daily living
- Inability to cope at home
- Poor or worsening general condition
- Already receiving home oxygen therapy
24
Q
why do we look for CO2 retention in patients with COPD
A
Excessive oxygen administration can lead to type 2 respiratory failure in patients with chronic hypercapnia (CO2 retainers). It was previously thought that over-administering oxygen would cause a loss of hypoxic drive and lead to type 2 respiratory failure. This is not true and patients suffering an acute COPD exacerbation generally have a near-normal respiratory drive, regardless of if they are CO2 retainers or not.
The true reasons for this phenomenon are due to two factors:
Ventilation/perfusion (V/Q) mismatches:
- In patients with COPD, hypoxia leads to vasoconstriction in the lungs leading to changes in alveolar V/Q ratios. This is to optimise their gas exchange.
- Over-administering oxygen undoes this and increases blood flow to damaged parts of the lung, leading to an increased V/Q mismatch and more dead space (regions of the lung that are not involved in gas exchange)
The Haldane Effect
- Deoxygenated haemoglobin binds to CO2 with greater affinity than oxygenated haemoglobin.
- Over-oxygenation causes a shift of the CO2 dissociation curve to the right – this is the Haldane effect.
- Patients with COPD may not be able to increase their respiratory rate to compensate for the increased amounts of dissolved CO2.
25
management of COPD exacerbation in primary care
**First line** increase dose/frequency of short-acting bronchodilators + 30 mg oral prednisolone for 5 days
+- **Give antibiotics** if there are clinical signs of pneumonia or the sputum is purulent:
**Options are**: amoxicillin, clarithromycin, or doxycycline
26
Medical treatment of COPD exacerbation
**First line**
* Salbutamol and ipratropium nebulisers and Hydrocortisone (IV) or prednisolone (PO)
* Antibiotics: If sputum purulent/clinical signs of infection
Chest physiotherapy to help sputum
**Second line**
* IV aminophylline
**Third line**:
* if pH is between 7.25-7.35: BiPAP
* if pH is below 7.25: intubationand ventilation
All patients given 30mg of pred for 5 days
27
Bronchiectasis
Bronchiectasis is a persistent or progressive chronic lung condition characterised by the permanent dilation of the bronchi due to permanent damage to the bronchial wall elastic and muscular tissue. This leads to copious sputum production, bacterial colonisation, and recurrent infections.
28
Pathophysiology of bronchiectasis
Bronchiectasis is caused by chronic inflammation of the airways and may develop after lung infections in childhood and in association with other conditions such as immunodeficiency or cystic fibrosis.
- TB
- Pertussis
- HIV
- Conenctiev tissue disorder e.g. SL
- CF
- Kartageners syndrome (primary ciliary dyskinesia- think infertility)
- Alpha-1- antitrypsin def
- Brocnhial obsturction e.g. tumours
29
Presentation of bronchiectasis
Patients typically have a chronic cough with copious amounts of purulent sputum. Features are:
* **Cough** – associated with large amounts of purulent sputum. There may be mild haemoptysis (small flecks of blood)
* **Crackles** on auscultation: Low-pitched crackles (rhonchi) are heard which are often cleared with coughing
* Shortness of breath
* Recurrent episodes of fever
* Weight loss
* Finger clubbing – this is rare in bronchiectasis
Features of an acute exacerbation:
* Recurrence of fever
* Increased sputum production
* Change in colour of sputum
30
investigations for bronchiectasis
**Chest x-ray:** Should be done in all patients as a baseline
* Ring or tubular opacities
* Tramlines
**High-resolution CT chest:** Gold-standard for diagnosis
* Thickened and dilated airways
* A ‘signet ring’ sign
**Sputum culture and sensitivity:**
* Haemophilus influenzae – most common
* Pseudomonas aeruginosa
31
management of bronchiectasis
The damage to the lungs cannot be reversed. Management involves preventing or slowing down further deterioration.
* Regular follow-up in secondary care
* Chest physio
* Lifestyle changes and measures (see patient advice)
* Influenza and pneumococcal vaccination
* Postural drainage and airway clearance physiotherapy techniques
* Antibiotics in acute exacerbations
* Consider surgery if there are areas of localised disease
32
Idiopathic Pulmonary Fibrosis
Lung fibrosis is where the interstitium of the lungs is replaced with scar tissue, leading to less elasticity and expandability in the lungs (reduced compliance), giving rise to the signs and symptoms seen.
It can happen secondary to lung damage (e.g. an acute infection such as pneumonia or tuberculosis), exposure to irritants (e.g. coal dust), or it can be idiopathic (IPF).
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causes of lung fibrosis mnemonic
Upper zone fibrosis: CHAARTSS
Lower zone fibrosis: RASIO
34
Upper zone fibrosis causes
Can be remembered using the mnemonic CHAARTSS:
Coal worker’s pneumonoconiosis
Histiocytosis
Ankylosing spondylitis
Allergic bronchopulmonary aspergillosis
Radiation
Tuberculosis
Sarcoidosis
Silicosis
35
Lower zone fibrosis causes
Can be remembered using the mnemonic RASIO:
* Rheumatoid arthritis and similar connective tissue disorders (e.g. systemic lupus erythematosus)
* Asbestosis
* Scleroderma
* Idiopathic pulmonary fibrosis (IPF)
* Others – (drugs such as bleomycin, nitrofurantoin, amiodarone, methotrexate)
36
IPF RF
Risk Factors
Increased age
Smoking
Family history
Occupational exposure e.g. silica or asbestos exposure, and raising livestock/birds
37
Presentation IPF
* Progressively worsening shortness of breath, particularly on exertion:
* Usually, the most prominent symptom and usually worsens insidiously
* Dry cough
* Bibasal end-inspiratory fine crackles on auscultation
* Finger clubbing
* Weight loss, fatigue, and malaise
38
IPF investigations
**Chest x-ray:**
* May show basilar reticular opacification
**Spirometry and lung function tests**: restrictive picture:
* FEV1 normal or decreased
* FVC decreased
* FEV1/FVC ratio >0.7 (increased)
* Transfer factor (TLCO): decreased
**High-resolution CT chest:**
* Shows *ground-glass* or opacification or *honeycombing*
39
management of IPF
* Most patients have pulmonary rehabilitation
* Some medications may be used e.g. pirfenidone
* Supportive care such as oxygen and physiotherapy
40
Hypersensitivity Pneumonitis
Formerly called e**xtrinsic allergic alveolitis**, hypersensitivity pneumonitis (HP) is characterised by *non-IgE-mediated immune inflammation* leading to inflammation of the lung tissue in people who have been sensitised by repeated inhalation of triggering antigens (e.g. dust, animal dander etc.).
**Chronic HP can lead to lung fibrosis.**
41
causes of hypersensitivity pneumonitis
Risk Factors and Associations
* Farmer’s lung – due to exposure to mouldy hay and Saccharopolyspora rectivirgula
* Bird fancier’s lung – due to exposure to avian proteins e.g. pigeons and parrots
* Cheese-worker’s lung – due to exposure to cheese mould
* Chemical worker’s lung – due to exposure to compounds used in the manufacture of plastics and rubber
* Some drugs – nitrofurantoin and methotrexate
42
Presentation of hypersensitivity pneumonitis
**Acute hypersensitivity pneumonitis**
Symptoms usually start 4-8 hours after exposure and resolve quickly, usually within days.
* Shortness of breath
* Dry cough
* Fever
**Chronic hypersensitivity pneumonitis**
Symptoms usually start weeks to months after exposure. Features are:
* Shortness of breath
* Productive cough
* Weight loss
* Clubbing
43
investigations for hypersensitivity pnuemonitis
**Chest x-ray:**
* May show infiltrates or fibrosis
**CT chest:**
* May show ground-glass shadowing
**Spirometry and lung function testing:**
* Usually shows a restrictive pattern
* Transfer factor (TLCO): reduced
**Blood serology testing to causative antigen:**
* Usually shows the presence of IgG to specific antigen
**Full blood count (FBC) and differential:**
* Normal, and as this is a non-IgE-mediated process, there is no eosinophilia
44
hypersensitvity pneumonitis management
1st-line: avoid precipitating factor
Consider using corticosteroids
45
Acute respiratory distress syndrome (ARDS)
is characterised by **sudden-onset** widespread **inflammation** of the lungs leading to **non-cardiogenic pulmonary oedema** (oedema of the lungs secondary to acute damage to the alveoli, and not heart failure).
46
pathophysiology of ARDS
The inflammation leads to increased capillary permeability within the lungs, allowing fluid to leak out, leading to oedema.
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RF for ARDS
* Sepsis
* Trauma with shock
* Multiple transfusions
* Acute pancreatitis
* Hypovolaemic shock
* Pneumonia
* Gastric aspiration
* Burns and smoke inhalation
* Vaping
48
ARDS presentation
* Dyspnoea
* Low O2 saturation
* Hypotension (think TALI)
* Tachypnoea
* Tachycardia
* Cough
* Bilateral lung crackles
49
ARDS investigations
**Chest x-ray:**
* Show **bilateral** infiltrates
**Arterial blood gases:**
* PaO2/FiO2 (fraction of inspired oxygen) ratio ≤300
**Pulmonary capillary wedge pressure:**
* If there is diagnostic doubt, this can be performed to exclude a cardiogenic cause for the pulmonary oedema
* In ARDS, the pulmonary capillary wedge pressure is not elevated
50
management of ARDS
1st-line: admit to ITU + manage underlying cause + supportive therapy
Intubation and ventilation may need to be considered
51
sarcoidosis is a
multisystem condition of **unknown aetiology**
characterised by:
- non-caseating (without necrotic centre) granulomas
52
sarcoidosis RF
Risk Factors
* Age 20-40 years
* Family history
* Female sex
* African-American ethnicity
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sarcoidosis presentation
The presentation can vary widely depending on what sites are affected.
**Classical presentations in exams are:**
* Cough + erythema nodosum (tender, erythematous nodules on the lower limbs)
* Bilateral hilar lymphadenopathy and hypercalcaemia
Some features seen may be:
* Cough – usually dry
* Dyspnoea – gradual in onset
* Wheeze on auscultation
* Lymphadenopathy – usually axillary, cervical, inguinal, and submandibular
* Symptoms of uveitis:
* **Red, painful eye**
* Blurred vision
* Photophobia
* Erythema nodosum
* **Lupus pernio** – hardened, raised, purple lesions on the nose, cheeks, lips, and ears
Other possible features can be:
* Arthralgia
* Hepato- and/or splenomegaly
* Heart failure
* Facial nerve palsy
* Arrhythmia
* Heart block
* Headaches
* Seizures
54
sarcoidosis investigations
**Chest x-ray:**
* Can be normal
* May show ***bilateral hilar lymphadenopathy***
**Serum calcium:**
* Hypercalcaemia is often present due to the cells in the granulomas producing calcitriol
***Serum ACE:***
* Usually elevated
**Urea and electrolytes (U&Es):**
* May be deranged
**Tuberculin skin test (Mantoux):**
* Usually negative
**Erythrocyte sedimentation rate (ESR):**
* Usually raised
**Tissue biopsy:**
* Shows non-caseating granulomas
55
Pulmonary sarcoidosis radiographic staging
* Stage 0: normal chest x-ray
* Stage I: bilateral hilar lymphadenopathy
* Stage 2: bilateral hilar lymphadenopathy + pulmonary infiltrates
* Stage 3: pulmonary infiltrates only
* Stage 4: pulmonary fibrosis
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management of sarcoidosis
steroids - mild lung function does not need treatment
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when are oral steroids are indicated in patients with sarcoidosis:
* Parenchymal lung disease (stage 2 or 3 + symptomatic)
* Uveitis (eye involvement)
* Neurological involvement
* Cardiac involvement
* Hypercalcaemia
58
Complications of sarcoidosis
Complications occur depending on what organs are affected:
Respiratory:
* Infection
* Pulmonary fibrosis
* Pulmonary hypertension
Cardiac:
* Arrhythmia
* Cardiac failure
Ophthalmological:
* Uveitis
Dermatological:
* Chronic refractory skin disease
Neurological:
* Neuropsychiatric problems
* Cranial and peripheral nerve damage
* Strokes and transient ischaemic attacks
Other:
* Hypercalcaemia
* Nephrolithiasis
* Arthritis
