Respiratory Flashcards
Define pneumothorax
Presence of air in the pleural space causing partial or total collapsing of the lung
Describe a primary spontaneous pneumothorax
- no underlying lung disease
- rupture of apical pleural bleb (cyst)
- risk factors = male, smoker, tall, thin
Describe a secondary spontaneous pneumothorax
Known lung disease:
- COPD
- asthma
- interstitial lung disease
- CF
- lung cancer
Infection:
- PCP
- TB
Genetic predisposition:
- catamenial pneumothorax (thoracic endometriosis)
Describe a traumatic pneumothorax
Penetrating chest wall injury
Puncture from rib fracture
Rupture of bronchus/oesophagus
Describe an iatrogenic pneumothorax
Doctor induced
Risk with:
- pacemakers
- CT lung biopsies
- central line insertion
- mechanical ventilation
- pleural aspiration
What are the signs/symptoms of a pneumothorax?
May be asymptomatic
Breathlessness
Tachypnoea
Pleuritic chest pain
Cough
Hypoxia/cyanosis
Unilateral chest wall expansion
Reduced breath sounds
Hyper-resonant percussion
* tension pneumothorax (emergency):
- deviated trachea
- surgical emphysema
- distended neck veins
- cardiovascular compromise
What investigations are needed for a pneumothorax?
CXR: shows collapsed lung
ABG: detect hypoxia
What are the treatments for a pneumothorax?
High flow oxygen
Conservative management (for small pneumothorax)
Aspiration
Chest drain
Surgery (for persistent/recurrent air leak)
*tension pneumothorax requires emergency needle decompression
Define pleural effusion
Collection of fluid in the pleural space
How are pleural effusions classified?
Transudates = pleural fluid protein < ½ serum protein
Exudates = pleural fluid protein > ½ serum protein
What are the causes of a transudate pleural effusion?
Increased hydrostatic pressure or reduced osmotic pressure in microvascular circulation
- heart failure
- cirrhotic liver disease
- renal failure
- hypoalbuminaemia
- ascites/peritoneal dialysis
What are the causes of a exudate pleural effusion?
Increased capillary permeability and impaired reabsorption
- pneumonia
- cancer
- TB
- autoimmune conditions (SLE, RA)
- PE
- drug induced (e.g. beta-blockers, methotrexate)
What are the signs/symptoms of pleural effusion?
Can be asymptomatic
Breathlessness
Cough
Pain
Fever
Reduced chest wall expansion
Quiet breath sounds
“Stoney” dull percussion
Tactile vocal fremitus/reduced resonance
What investigations are needed for pleural effusion?
CXR: small = blunt costophrenic angles, large = water-dense shadows with concave upper borders
Ultrasound: identifies fluid
Aspiration: DIAGNOSTIC, tested for protein, glucose, pH, microscopy & culture, immunology (e.g. rheumatoid factor)
Biopsy: is aspiration is inconclusive
What are the treatments of pleural effusion?
Conservative management for small effusions
Drainage (may need repeating)
Pleurodesis (uses talc to treat recurrent effusions)
Describe a haemothorax
Blood in the pleural cavity
Caused by trauma, post-operative, bleeding disorders, lung cancer, PE
Treated with chest drain, vascular intervention, surgery
Describe a hydropneumothorax
Air and fluid in the pleural space
Causes: iatrogenic, thoracic trauma, gas forming organisms
CXR: perfectly straight fluid level
Describe empyema
Pus in the pleural space
Patient has resolving pneumonia and develops a recurrent fever
CXR: pleural effusion
Aspiration: yellow and turbid, low pH, low glucose
Treated with chest drain
Define COPD
Chronic obstructive pulmonary disease, characterised by persistent respiratory symptoms (such as breathlessness, cough, and sputum) and airflow obstruction (usually progressive and not fully reversible)
What causes COPD?
Bronchitis (clinical = cough, sputum production) + emphysema (histological = destruction of alveolar walls, enlarged air spaces)
Chronic inflammation caused by exposure to noxious particles or gases (usually tobacco smoke but also from environmental and occupational exposures)
Describe the epidemiology of COPD
3rd highest cause of death globally
Prevalence is equal in men and women
Onset > 35 y/o
Associated with smoking or pollution
What are the risk factors for COPD?
Smoking
Occupational exposures (dusts, fumes, chemicals)
Air pollution
Genetic factors (alpha-1 antitrypsin)
Lung development in-utero/childhood
What are the signs/symptoms of COPD?
Shortness of breath
Cough
Sputum production
Wheeze
Tachypnoea
Orthopnoea
Accessory muscle use
Hyperinflation of chest
Cyanosis
Cor pulmonale (raised JVP, peripheral oedema)
Weight loss
What investigations are needed for COPD?
Spirometry: FEV1/FVC ratio < 0.7 or 70% (little or no reversibility after bronchodilator)
CXR: hyperinflated lungs, excludes other causes
CT: more detail of structural damage (bronchial wall thickening, increased air spaces)
Serum alpha-1-antitrypsin: detects deficiency
FBC: may show anaemia, high platelets
What are the investigations needed for an exacerbation of COPD
ABG
CXR
ECG
FBC
ESR/CRP
Sputum culture
What are the treatments for COPD?
Non-pharmacological:
- smoking cessation
- physiotherapy
- pulmonary rehabilitation
- psychological support
- optimise comorbidities
Pharmacological:
- 1st = short-acting beta agonist (e.g. salbutamol) or muscarinic antagonist (e.g. ipratropium) as a reliever
- 2nd + long-acting beta agonist (e.g. salmeterol) and muscarinic antagonist (e.g. tiotropium), OR + inhaled corticosteroid (e.g. beclomethasone) if asthmatic features
- 3rd = LABA + LAMA + ICS
- 4th = refer to specialist (oral steroids, theophylline, mucolytics, prophylactic antibiotics, long-term oxygen)
What are the treatments of an acute exacerbation of COPD?
Nebulised bronchodilators (salbutamol + ipratropium)
Oxygen therapy (with care, as some patients rely on hypoxic drive)
Steroids: IV hydrocortisone + oral prednisolone
Antibiotics (if infectious cause)
IV theophylline
Intubation/ventilation
Describe the epidemiology of lung cancer
2rd most common cancer
More common in males
Incidence is decreasing
10% survival rate after 10 years
What are the causes of lung cancer?
Cigarette smoking (most common, including passive smoking)
Occupational - asbestos, nickel, arsenic
Lung fibrosis
What are the signs/symptoms of lung caner?
Local:
- cough
- shortness of breath
- haemoptysis
- chest pain
- recurrent chest infections
Systemic:
- general malaise
- weight loss
- paraneoplastic syndrome
- clubbing
- anaemia
What are the types of lung cancer?
Primary carcinoma: (95% are bronchial carcinomas)
- small cell (neuroendocrine tumours, often cause paraneoplastic syndromes)
- non-small cell (squamous cell, adenocarcinoma, or large cell)
Metastatic carcinomas (more common than primary carcinomas)
Others:
- benign tumours
- salivary gland type tumours
- soft tissue tumours
- lymphoma
What investigations are needed for lung cancer?
Bloods: FBC (anaemia), LFTs and U&Es (effect of metastases)
Imaging: CXR, CT
Biopsy (DIAGNOSTIC)
Molecular testing
What are the treatment of lung cancer?
Surgery
Chemo/radio therapy
Palliative care
What are the types of tumours of the pleural?
Pleural fibroma (benign)
Mesothelioma (malignant, caused by asbestos)
Define occupational lung disease
A wide-range of respiratory conditions caused by inhaling a harmful substance in the workplace or exposures which may aggravate the symptoms of any pre-existing lung disease
What things may cause occupational lung diseases?
Dusts - chiselling stone/cutting wood, flour
Fumes - soldering/welding
Mists - coolant used in metal work
Vapours/gases - spray paint/gas leaks
What are the most common occupational lung diseases?
Asthma
Non-malignant pleural disease
Pneumoconiosis
Inhalation accidents
Bronchitis/emphysema
Lung cancer
Mesothelioma
What factors affect a persons development of occupational lung diseases?
The physical and chemical nature of the agent
The duration and dose of exposure
Individual susceptibility
Describe the different progressions of occupational lung disease
- Immediate effects: e.g. high dose exposure to chlorine gas resulting in acute airway injury and chronic asthma
- Short latency (months-years): present while patient is still exposed e.g. occupational asthma
- Long latency (often decades): commonly present close to/after retirement e.g. asbestosis, silicosis
How are occupational lung diseases managed?
Standard lung disease education and treatment
Liaison with occupational health and/or employer
Compensation advice
Prevention or reduction of exposure
What are the effects of occupational lung disease?
Unemployment (up to one third of OA)
Loss of earnings
Chronic resp ill health
Depression
Loss of self worth
Breakdown of relationship
How are occupational lung diseases prevented?
Elimination or substitution of cause
Engineering controls (ventilation)
Training and behaviour
Respiratory protective equipment
Surveillance of workers at risk
What are the common causes of occupational asthma?
Flour
Cleaning products/disinfectant
Isocyanates (in spray paint)
Glue/adhesive
Wood
Welding fumes
Define hypersensitivity pneumonitis
Granulomatous inflammation (due to type III hypersensitivity reaction) of the lung parenchyma and airways due to repeated inhalation of organic antigens in dusts (e.g. from dairy/grain products, animal dander, chemicals)
What are the different forms of hypersensitivity pneumocystis?
Acute:
- 4-8 hours after exposure, resolves quickly
- flu-like illness = fever, chest tightness, dry cough, dyspnoea
Subacute:
- gradual onset, may be history of repeated attacks
- less severe symptoms = productive cough, dyspnoea, fatigue
Chronic:
- long-term exposure, gradual onset
- exercise intolerance, weight loss, may develop cyanosis, clubbing, hypoxaemia, right-sided heart failure
- can cause fibrosis, emphysema, and permeant damage
What are the causes of pneumoconiosis?
Asbestos = pleural disease -> lung fibrosis (asbestosis) -> cancer (mesothelioma)
Silica (from stone/brick) = causes silicosis, COPD, can lead to cancer
Coal dust = known as coal worker’s pneumoconiosis
Define asthma
A chronic respiratory condition associated with inflammation and airway hyperresponsiveness, leading to episodic bronchospasm due to triggers
Describe the epidemiology of asthma?
Incidence is higher in children
More common in boys in early childhood, but more common in female adults
Occupational asthma accounts for 15% of adult onset asthma
Describe the pathophysiology of asthma
Dendritic cells recognise and present allergen peptides to T-cells which release cytokines to activate other immune cells (such as mast cells which release histamine on IgE binding), resulting in contraction of the airway smooth muscle cells, causing reversible obstruction or bronchospasm in exacerbations
What are the risk factors for asthma?
Personal/family history of atopic disease (asthma, eczema, allergic rhinitis)
Male sex (children)/female sex (adult)
Premature birth/low birth weight
Obesity
Social deprivation (exposure to damp housing, pollution, smoking)
Workplace exposures (dust, spray paints etc.)
Exposure to allergens at home (pets, feather pillows/duvets etc.)
What are the sings/symptoms of asthma?
Breathlessness
Wheezing (non-specific)
Cough
Chest tightness
Nocturnal symptoms
What things can trigger asthma?
Exercise
Allergen
Irritant exposure
Changes in weather
Respiratory infections
Work-related triggers (e.g. dust, spray paint etc.)
Smoking/passive smoking
Pollution
Emotional stress
Drugs (NSAIDs, beta-blockers)
What are the investigations needed for asthma?
Spirometry: FEV1/FVC < 0.7 (obstructive), or seen during bronchial challenge, reversible by 12% after using bronchodilator
Peak flow meter: taken x2 daily for 1-2 weeks, shows 20% diurnal variability
Detect allergens: measure total/specific IgE in blood, or skin prick testing
FeNO testing (detects eosinophilic airway inflammation)
What are the treatments for asthma?
Non-pharmacological:
- smoking cessation
- weight loss
- physio/speech therapy
- allergen/air pollutant avoidance
Pharmacological:
- 1st = reliever inhaler of short-acting beta-agonist (e.g. salbutamol)
- 2nd + low-dose inhaled corticosteroid (e.g. beclomethasone)
- 3rd + leukotriene receptor antagonist (e.g. montelukast)
- 4th + long-acting beta-agonist (e.g. salmeterol) +/- LTRA
- 5th = specialist input (+ oral steroids/theophylline/biologics)
What are the emergency treatments for an acute asthma attack?
Nebulised bronchodilators (e.g. salbutamol + ipratropium if severe)
Oxygen (if low sats)
Systemic steroids (oral prednisolone/IV hydrocortisone)
IV magnesium sulphate (if severe, anti-inflammatory and bronchodilator)
Antibiotics (only if infective cause)
Intubation/ventilation
How do the treatments for asthma/COPD work?
- beta agonists = relax bronchial smooth muscle by increasing cAMP, acting within minuets (short acting) or overnight (long acting)
- corticosteroids = decrease bronchial mucosal inflammation, acting over days
- leukotriene receptor antagonists = block the effects of cysteinyl leukotrienes in the airways to reduce inflammation
- muscarinic antagonists = decrease bronchial smooth muscle spasm, can be short or long acting (used for COPD not asthma)
- theophylline = inhibits phosphodiesterase, so decreases bronchoconstriction by increasing cAMP
- biologics (e.g. omalizumab) = anti-IgE monoclonal antibody
What are the common causes of upper respiratory tract illnesses?
Viral illness:
- Rhinoviruses (45-50%)
- Influenza A virus (25-30%)
- Coronaviruses (10-15%)
- Adenoviruses (5-10%)
- Respiratory Syncytial viruses (5%, immunocompromised or children )
What are the complications of upper respiratory tract illnesses?
May lead to bacterial infection
May causes systemic symptoms (particularly influenza A and SARS-CoV2)
Can lead to sinusitis, pharyngitis, bronchitis, pneumonia (rare)
What are the causes of pharyngitis?
Viral (70-80%):
- rhinovirus, adenovirus etc.
- EBV, acute HIV
Bacterial:
- S. pyogenes (Lancefield group A, b-haemolytic, treatment = amoxicillin)
- Corynebacterium diphtheriae (very rare, greyish membrane on tonsils, treatment = antitoxin, erythromycin/clarithromycin)
- Neisseria gonorrhoea (and other sexually transmitted infections)
- Mycoplasma pneumoniae (occurs in epidemics)
Describe bronchitis
Inflammation of the bronchi epithelium due to upper airway infection
Causes: mostly viral
Symptoms: cough (1-3 weeks), SOB, wheeze, no systemic features
Diagnosis: viral swab for PCR, CXR = normal
Treatment: usually none (self-limiting)
Describe Haemophilus influenzae
A gram negative coccobacilli
Encapsulated form = Haemophilus type B (HiB)
Can cause acute epiglottitis
Treated with co-amoxiclav/doxycycline (most are are beta-lactamase producers)
Describe Bordatella pertussis
Gram negative bacilli, cause of whooping cough
Diagnosis = culture, PCR, or ELISA for IgG against pertussis toxin
Clinical course = incubation, catarrhal phase (rhinorrhoea, fever, conjunctivitis), paroxysmal phase (coughing spasms, inspiratory whoop, vomiting)
Treatment = clarithromycin, vaccination
What is a multiplex PCR?
Used to diagnose respiratory viruses
Green viral throat swab
RNA/DNA extracted and amplified by PCR using probes for each of the common viruses
Results within 24 hours
Define pneumonia
Inflammation of the lung parenchyma caused by infections (usually bacteria)
Describe the epidemiology of pneumonia
High incidence and hospitalisation
High cost to NHS
Usually only in those at risk
Significant short and long term mortality
What are the risk factors for pneumonia?
Infants and the elderly
COPD and certain other chronic lung diseases
Immunocompromised
Nursing home residents
Impaired swallow (neurological conditions etc.)
Diabetes
Congestive heart disease
Alcoholics and intravenous drug users
What are the typical organisms which cause pneumonia?
Streptococcus pneumoniae (most common)
Haemophilus influenzae
Moraxella catarrhalis
Staphylococcus aureus
Klebsiella pneumoniae
Viruses
Describe some atypical pathogens which causes pneumonia
Legionella (from warm water, causes severe illness)
Mycoplasma pneumoniae (epidemic, causes extra-pulmonary features e.g. rash)
Chlamydia pneumoniae (often older adults with closed outbreaks, longer duration of symptoms)
- diagnosis = needs serology (intracellular so difficult to grow)
- treatment = erythromycin/clarithromycin (not susceptible to beta-lactams)
Describe the pathophysiology of pneumonia
Bacteria colonises in the airway and overwhelms the respiratory host defences, developing an inflammatory response, where inflammatory exudate/pus (dead bacteria + dead neutrophils + tissue fluid) fill the alveolar space
What are the signs/symptoms of pneumonia?
Local:
- cough
- sputum (rusty sputum suggests S. pneumoniae)
- shortness of breath
- pleuritic chest pain
- tachypnoea
- signs of consolidation (reduced expansion, dull percussion, increased vocal resonance)
Systemic:
- malaise
- weakness
- fever, sweats, rigors
- tachycardia
- hypotension
- hypoxia, cyanosis
What are the red flags for a respiratory infection?
Delirium/confusion
Renal impairment (high urea)
Increased oxygen demand (high resp rate, high lactic acid from anaerobic respiration)
Low blood pressure
*all signs of sepsis
What investigations are needed for pneumonia?
FBC: raised WBC
U&E: asses renal function for severity
CRP/ESR: asses severity of inflammation
Oxygen: pulse oximetry, ABG (define respiratory failure
Microbiology testing: sputum, urine for legionella, pleural fluid (using bronchoalveolar lavage if needed), PCR (viruses)
CXR: infiltrates, pleural effusion, or cavitation
What is the CURB65 score?
Asses the severity of community-acquired pneumonia
One point for each:
- confusion
- urea > 7mmol/L
- respiratory rate > 30/min
- blood pressure < 90/60
- age > 65
0 = mild
1-2 = moderate
3-5 = severe
Predicts mortality and need for admission
What is the empirical treatment for pneumonia?
Mild severity (CURB65 = 0-1):
- oral amoxicillin or clarithromycin if allergic
Moderate severity (CURB65 = 2):
- oral amoxicillin + clarithromycin
Severe (CURB65 = 3-5):
- IV co-amoxiclav/cefuroxime + clarithromycin
What antibiotics are used to treat pneumonia caused by the following bacteria:
- S. pneumoniae
- H. influenzae
- Staph. aureus
- Klebsiella pneumoniae
- Legionella pneumoniae
S. pneumoniae = amoxicillin/cefuroxime
H. influenzae = co-amoxiclav/doxycycline
Staph. aureus = flucloxacillin/vancomycin (if MRSA)
Klebsiella pneumoniae = co-amoxiclav/cefuroxime
Legionella pneumoniae = fluoroquinolone + clarithromycin
How is pneumonia prevented?
Vaccine: e.g. polysaccharide pneumococcal vaccine
- protects against 23 serotypes
- given to adults >65 y/o, immunocompromised, and co-morbid patients
Smoking cessation also helps
Describe hospital acquired pneumonia
Acquired at least 48 hours after hospital admission
Most commonly in elderly, patients on ventilators, or post operative
Likely to be more resistant organisms and harder to treat = needs broader/complex antibiotics (e.g. metronidazole, doxycycline, piperacillin-tazobactam)
What are the common causes of hospital acquired pneumonia?
Gram negative enterobacteria
Staphylococcus aureus
Pseudomonas aeruginosa
Klebsiella pneumoniae
Bacteroides
What are the common causes of pneumonia in immunocompromised patients?
All typical bacteria
Fungal: pneumocystis jirovecii (treated with co-trimoxazole)
Viral: cytomegalovirus, RSV, herpes
Describe the epidemiology of TB
Leading cause of death from a single infectious agent (above HIV/AIDS)
Mostly in Southern Africa and South East Asia
Associated with HIV
How is TB spread?
Most: aerosol from infected individuals entering the lungs
Rare: drinking unpasteurised milk from a cow infected with M. bovis (gut TB)
Describe the pathophysiology of TB
Infection is caused by Mycobacterium tuberculosis (active TB)
The bacilli contaminate macrophages and T lymphocytes and remain while the initial infection is cleared (latent TB)
A granuloma (most commonly in upper lobe) persists and develops into a cavity, with other nodes being affected, and causing effusion/collapse
There can also be haematogenous dissemination, leading to serious non-pulmonary disease (e.g. miliary TB, GI/GU TB, spinal TB etc.)
What are the signs/symptoms of TB?
Pulmonary:
- cough
- chest pain
- breathlessness
- haemoptysis
Systemic:
- weight loss
- night sweats
- malaise, fever, anorexia
What are the investigations needed for active TB?
Non-specific: FBC = anaemia, ESR/CRO = raised
Microscopy, culture (sputum)
PCR
Biopsy of lymph nodes/affected structures
CXR: fibronodular/linear opacities in upper lobes, effusion, lymphadenopathy
What are the investigations needed for latent TB?
Cannot culture bacteria
Tuberculin skin test/Mantoux test: antigen injected intradermally, fast inflammatory response shows T-cell memory, indicating previous exposure
Interferon gamma release assays (IGRAs): stimulates memory T cells with specific TB antigens (no vaccine)
What are the treatments of active TB?
4 drugs for 6 months: (RIPE)
- rifampicin ‘R’ (6m) SE = hepatitis, red urine/sweat/tears
- isoniazid ‘H’ (6m) SE = hepatitis, peripheral neuropathy
- pyrazinamide ‘Z’ (2m) SE = hepatitis, rash
- ethambutol ‘E’ (2m) SE = optic neuritis
What are the treatments for latent TB?
Isoniazid (6m)
Rifampicin + isoniazid (3m)
How is TB prevented?
Active case finding – reduce infectivity
Detection and treatment of latent TB
Neonatal BCG vaccination
Describe drug resistant TB
Drug resistance may be to single or multiple drugs
Risk factors: previous TB treatment, poor quality drugs, non-adherence
Common in former soviet union, china
What ABG results are seen in type 1 respiratory failure?
O2 = low
CO2 = normal/low
HCO3 = normal
What ABG results are seen in type 2 respiratory failure?
O2 = low
CO2 = high
HCO3 = normal (acute), high (chronic)
What are some common causes of type 1 respiratory failure?
Pneumonia
Pulmonary embolism
Altitude
Lung fibrosis
What are some common causes of type 2 respiratory failure?
Obstruction (COPD/severe asthma)
Muscle weakness (motor neuron disease)
Reduced respiratory drive (e.g. opiate SE)
Obesity
Define cystic fibrosis
A multi-organ disease caused by an abnormality in the CFTR gene, causing respiratory, gastrointestinal, biliary, and pancreatic problems
Describe the epidemiology of cystic fibrosis
Most common inherited disease in Caucasian population
Only risk factor is family history
All UK babies are screened at birth
Describe the pathophysiology in the lungs of cystic fibrosis
CFTR is chloride channel that also affects other cellular activities, like sodium transport in respiratory epithelium, composition of cells surface glycoprotein, and antibacterial defences
Defects in these processes cause reduced mucociliary clearance and increased bacterial colonisation, leading to inflammatory lung damage
What are the signs/symptoms of cystic fibrosis?
Neonates:
- meconium ileus
- failure to thrive
- rectal prolapse
- prolonged jaundice
Children/adolescents:
- cough
- wheeze
- recurrent respiratory infections
- nasal polyps
- bronchiectasis
- pancreatic insufficiency
- portal hypertension/variceal haemorrhage
- male infertility
- finger clubbing
What are the investigations needed for cystic fibrosis?
Sweat testing (DIAGNOSTIC): high chloride, low sodium, 98% sensitive
Molecular genetic testing for CFTR
Bloods: FBC, U&Es, LFTs, vitamins A, D and E, glucose
Sputum culture: common pathogens include Pseudomonas aeruginosa, H. influenzae, S. aureus
CXR: dilated airways filled with mucus, detect pneumonia/pneumothorax
Abdominal ultrasound: fatty liver, chronic pancreatitis
Sinus x-ray/CT: opacification of sinuses
Spirometry: obstructive defect
What are the treatments for cystic fibrosis?
Chest physiotherapy (postural drainage, airway clearance techniques)
Antibiotics: for infective exacerbations, and prophylactically
Mucolytics (e.g. Dornase alfa): cleaves neutrophil-derived DNA to reduce viscosity and aid removal of sputum
Pancreatic enzyme replacement
Maintain adequate weight (high energy meals)
Screening and treatment for diabetes and osteoporosis
Psychological interventions
Heart/lung transplant in severe disease
Define bronchiectasis
Chronic inflammation of the bronchi and bronchioles leading to permanent dilatation and thinning of these airways
Describe the epidemiology of bronchiectasis
Prevalence increases with age
Slightly more common in women
What are the causes of bronchiectasis?
Previous severe lower respiratory tract infection:
- pneumonia
- pertussis
- pulmonary TB
- influenza (most common)
Congenital:
- cystic fibrosis
- primary ciliary dyskinesia
- Young’s syndrome
Associated conditions:
- COPD
- asthma
- rheumatoid arthritis
- IBD
- allergic bronchopulmonary aspergillosis
Obstruction:
- bronchial tumour
- foreign body
Describe the pathophysiology of bronchiectasis
Irreversible inflammatory damage to the elastic and muscular components of the bronchial walls causes the permanent dilatation
What are the signs/symptoms of bronchiectasis?
Persistent cough
Copious purulent sputum
Haemoptysis
Dyspnoea
Wheeze
Coarse crackles
Finger clubbing
Systemic: fever, fatigue, weight loss
What are the investigations needed for bronchiectasis?
Sputum culture: often colonise Pseudomonas aeruginosa, or S. aureus
FBC: raised WBC in infection
CXR: exclude other pathology, may show abnormalities in severe disease
High-resolution CT: DIAGNOSTIC, asses extent and distribution of disease
Spirometry: often shows obstructive pattern, may show reversibility
Immunoglobulins: test for IgG, IgA, and IgM deficiency
Bronchoscopy: exclude obstruction, obtain sample for culture
Other tests: CF testing, IgE/skin prick test for Aspergillus, RF/anti-CPP/ANCA for RA
What are the treatments for bronchiectasis?
Physiotherapy:
- airway clearance
- exercise
Antibiotics:
- for acute exacerbations, depends on sensitivities (e.g. Pseudomonas = oral ciprofloxacin)
- long-term, for recurrent exacerbations, depends on sensitivities
Bronchodilators:
- beta agonists (e.g. nebulised salbutamol)
- muscarinic antagonists
- theophylline
- corticosteroids (only if also asthmatic)
Surgery: (in severe disease)
- resection
- bronchial artery embolisation
Define interstitial lung disease
A heterogenous group of conditions, which are characterised by scarring (fibrosis) and inflammation of the lung interstitium
How are interstitial lung diseases categorised?
Idiopathic:
- idiopathic pulmonary fibrosis
- non-specific interstitial pneumonitis
Known cause:
- occupational/environmental (e.g. asbestosis, silicosis)
- drug-associated (e.g. nitrofurantoin)
- hypersensitivity pneumonitis
- infection (e.g. TB, fungi, viral)
Associated with systemic disorders:
- sarcoidosis
- rheumatoid arthritis
- SLE
- systemic sclerosis
What are the shared clinical features of interstitial lung disease?
Dyspnoea (on exertion)
Non-productive coughing
Insidious fatigue and weight loss
Abnormal breath sounds
Abnormal CXR or HRCT
Restrictive pattern spirometry
Decreased TLCO
Define idiopathic pulmonary fibrosis
The most common type of interstitial lung disease, characterised by fibrosis and inflammatory cell infiltrates of unknown cause
Describe the pathophysiology of idiopathic pulmonary fibrosis
Proliferation of fibroblasts (forming foci) which deposit collagen in the extracellular matrix, causing thickened alveolar interstitium, destruction of alveoli, and lower gas exchange, mostly affecting the periphery and base of lungs
What are the treatments for idiopathic pulmonary fibrosis
Supportive care: oxygen, opioids analgesics, palliative care
Pirfenidone (inhibits fibroblasts + immunosuppressive) and nintedanib (inhibits growth factor receptors) to reduce the rate of scarring and slow disease progression
Define sarcoidosis
A multi-system chronic granulomatous inflammatory disorder of unknown cause, commonly affecting the lungs and thoracic cavity but also other sites (e.g. skin, eyes, liver, nervous system)
Describe the epidemiology of sarcoidosis
Commonly affects 20-40 y/o
More common in women
More common and severe in Afro-Caribbeans than Caucasians
What are the clinical features of sarcoidosis?
Pulmonary:
- dry cough, dyspnoea, fever, chest pain
- abnormal CXR (bilateral hilar lymphadenopathy +/- peripheral infiltrates)
- restrictive/obstructive pattern
Non-pulmonary:
- raised serum ACE
- rash
- uveitis
- lymphadenopathy
- hepatosplenomegaly
- Bell’s palsy, neuropathy, meningitis
- cardiomyopathy, arrhythmias
What are the treatments of sarcoidosis?
Only early pulmonary disease = bed rest, NSAIDs
Corticosteroids (oral prednisolone)
Immunosuppressants (e.g. methotrexate)
Anti-TNF (e.g. adalimumab)
