Bronchiectasis, CF, Lung Ca and Asthma Flashcards
Bronchiectasis - Definition
Chronic infection of the bronchi and bronchioles leading to permanent dilation of these airways.
The main organisms are Strep pneumonia, Haem influenza, Staph aureus or Pseudomonas aeruginosa.
Bronchiectasis - Causes
Congenital – cystic fibrosis, Young’s syndrome (triad with rhunosinusitis and reduced fertility), primary ciliary dyskinesia and Kartagener’s syndrome (triad with sinusitis and dextracardia).
Post-infection – measles, pertussis, bronchiolitis, pneumonia, TB or HIV.
Other – bronchial obstruction (tumour, foreign body), allergic bronchopulmonary aspergillosis (ABPA), hypogammglobulinaemia, rheumatoid arthritis, ulcerative colitis or idiopathic.
Bronchiectasis - clinical features
- Symptoms – persistent cough with copious purulent sputum and intermittent haemoptysis.
- Signs – finger clubbing, coarse inspiratory crepitations and wheeze.
- Complications – pneumonia, pleural effusion, pneumothorax or a cerebral abscess.
Bronchiectasis - investigations
Sputum for culture and sensitivity, CXR will show thickened bronchial walls and dilated airways, spirometry which will show an obstructive pattern and can help determine level of reversibility and bronchoscopy to locate the site of haemoptysis or exclude an obstruction.
Bronchiectasis - management
Postural drainage – should be performed twice daily and chest physiotherapy is also used.
Antibiotics – these should be prescribed according to the bacterial sensitivities.
Bronchodilators – e.g. nebulised salbutamol may be useful in patients with asthma or COPD.
Surgery – may be indicated in localised disease or in order to control severe haemoptysis.
Cystic fibrosis - definition
Affects 1 in 2000 live births – one of the most common life-threatening autosomal recessive conditions affecting Caucasians.
It is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene (chromosome 7) which encodes a chloride channel.
This results in changes in the composition of airway surfaces lipid and predisposes the lung to chronic pulmonary infections and bronchiectasis.
Cystic fibrosis - clniical features
Neonates – failure to thrive, meconium ileus (the ileum is congested) or rectal prolapse.
Children:
- Respiratory – cough, wheeze, recurrent infections, bronchiectasis, spontaneous pneumothorax, haemoptysis, respiratory failure or cor pulmonale.
- Gastro – pancreatic insufficiency (diabetes mellitus and steatorrhoea), distal intestinal obstruction syndrome (meconium ileus equivalent), gallstones or cirrhosis.
- Other – male infertility, osteoporosis, arthritis, vasculitis, nasal polyps, sinusitis and hypertrophic pulmonary osteoarthropathy.
- Signs – finger clubbing, peripheral and central cyanosis and bilateral coarse crackles.
Cystic fibrosis - diagnosis
Sweat test – sodium and chloride are >60mmol/L, genetics – screening for common CF mutations should be performed and faecal elastase – for decreased exocrine pancreatic function.
Cystic fibrosis - investigations
Bloods – FBC, Us + Es, LFTs, clotting, vitamin A, D and E levels and annual glucose tolerance tests.
Bacteriology – sputum culture to detect respiratory infections.
Chest x-ray – to look for hyperinflation and bronchiectasis.
Abdominal ultrasound – fatty liver, cirrhosis and chronic pancreatitis.
Spirometry – will reveal an obstructive picture.
Biochemistry – for faecal fat analysis.
Cystic fibrosis - management
With a MDT including a physician, GP, specialist nurse, physiotherapist and dietician:
- Chest – regular physiotherapy – postural drainage, active cycle breathing techniques or forced expiratory techniques, antibiotics – for acute infective exacerbations and prophylactically, mucolytics e.g. 2.5mg Dornase alfa nebulised daily and bronchodilators e.g. salbutamol.
- Gastrointestinal – pancreatic enzyme replacement, fat soluble vitamin supplements (A, D, E and K), ursodeoxycholic acid for impaired liver function or transplantation for cirrhosis.
- Other – treatment for CF related diabetes, screening for and treatment of osteoporosis, treatment for arthritis, sinusitis and vasculitis and fertility and genetic counselling.
- Advanced lung disease – O2, diuretics (cor pulmonale), non-invasive ventilation or transplant.
Cystic fibrosis - prognosis
The median survival for patients with cystic fibrosis is now over 30 years.
Bronchial carcinoma - epidemiology and risk factors
The most common type of lung malignancy – accounts for 19% of all cancers and 27% of all cancer deaths per year in the UK. The incidence is currently increasing in women in the UK.
Risk factors – cigarette smoking (the main one), asbestos, chromium, arsenic, iron oxides and radiation.
Bronchial carcinoma - histology
Squamous in 35%, adenocarcinoma in 27%, small cell in 20%, large cell in 10% and alveolar cell carcinoma in <1%.
Clinically the most important division is between small cell and non-small cell.
Bronchial carcinoma - staging
- Tumour – TX – malignant cells in bronchial secretions, TIS – carcinoma in situ, T0 – none, T1 - <3cm in lobar or distal airway, T2 - >3cm and >2cm distal to carina or T3 - <2cm distal to carina and T4 – involves the mediastinum, heart, great vessels, trachea, oesophagus or vertebrae.
- Nodes – N0 – none, N1 – peribronchial, N2 – ipsilateral and N3 – contralateral mediastinum.
- Metastases – M0 – none or M1 – distant metastases present.
Bronchial carcinoma - clinical features
- Symptoms – cough (80%), haemoptysis (70%), dyspnoea (60%), chest pain (40%), recurrent or slowly resolving pneumonia, anorexia and weight loss.
- General signs – cachexia, anaemia, clubbing, hypertrophic pulmonary osteoarthropathy (HPOA – new bone formation causes wrist pain) and supraclavicular and axillary nodes.
- Chest – can be no signs in the chest or consolidation, collapse or pleural effusion.
- Metastases – bone tenderness, hepatomegaly, confusion, fits or faints, focal neurological signs, cerebellar syndrome, proximal myopathy or peripheral neuropathy.
Bronchial carcinoma - complications
- Local – recurrent laryngeal nerve palsy, phrenic nerve palsy, superior vena cava obstruction, Horner’s syndrome (Pancoast’s tumour), rib erosion, pericarditis or atrial fibrillation.
- Metastatic – to brain, bone (bone pain, anaemia and high Ca+), liver or adrenals (Addison’s).
- Endocrine – ectopic hormone secretion e.g. SIADH (high ADH and low Na+), ACTH by small cell tumours causing Cushing’s and PTH by squamous cell tumours leading to hypercalcaemia.
- Neurological – confusion, fits, cerebellar syndrome, proximal myopathy, neuropathy, polymyositis or Lambert-Eaton syndrome (AI destruction of neuromuscular junctions).
- Other – clubbing, HPOA, dermatomyositis, acanthosis nigricans or thrombophlebitis migrans.
Bronchial carcinoma - investigations
- Cytology – sputum and pleural fluid to aid diagnosis – must send at least 20mL to the lab.
- Chest x-ray – for peripheral nodules, hilar enlargement, consolidation, collapse or effusion or bony secondary’s. Lesions or nodes may be suitable for fine needle aspiration or biopsy.
- Bronchoscopy – to give histology and assess operability.
- CT or 18F-deoxyglucose PET scan – to help stage tumour by looking for nodes and metastases.
- Radionucleotide bone scan – should be performed if bone metastases are suspected.
- Lung function tests – to assess patient reserve and suitability for lobectomy.
Bronchial carcinoma - management
- Non-small cell carcinoma – excision for peripheral tumours with no metastatic spread or chemotherapy (platinum based regimes) ± radiotherapy for more advanced disease.
- Small-cell carcinoma – may respond to chemotherapy ± radiotherapy but invariably relapse.
- Palliation – radiotherapy for bronchial obstruction SVC obstruction, haemoptysis, bone pain and cerebral metastases. Endobronchial therapies include tracheal stenting, cryotherapy, laser or brachytherapy (radioactive source is placed close to the tumour). Pleural drainage for pleural effusion.
- Drugs – analgesia, steroids, anti-emetics, bronchodilators or antidepressants.
Bronchial carcinoma - prognosis and prevention
Prognosis – non small-cell lung carcinoma – 50% 2 year survival without spread but 10% with spread. Small cell lung carcinoma – median survival is 3 months if untreated and 1 – 1.5 years if treated.
Prevention – encourage patients to quit smoking and prevent occupational exposure to carcinogens.
Bronchial adenoma
A rare slow growing tumour (90% carcinoid) that is treated with surgery.
Hamartoma
A rare, benign tumour with flecks of calcification – excise to exclude malignancy.
Mesothelioma - definition and clinical features
A tumour of mesothelial cells that usually occurs in the pleura and rarely the peritoneum or other organs. It is associated to occupational exposure to asbestos but the relationship is complex – latent period between exposure and development can be 45 years.
Clinical features – chest pain, dyspnoea, weight loss, finger clubbing, recurrent pleural effusions. There may also be signs of metastases – lymphadenopathy, hepatomegaly, bone pain or tenderness, abdominal pain or obstruction (peritoneal mesothelioma).
Mesothelioma - investigation and management
Investigations – chest x-ray or CT will show pleural thickening or pleural effusion.
Diagnosis – made on histology following pleural biopsy or only at post mortem.
Management – chemotherapy can improve survival but radiotherapy is controversial.
Prognosis – is poor and over 650 deaths per year occur in the UK.
Asthma - definition
Affects 5-8% of the population – characterised by recurrent episodes of dyspnoea, cough and wheeze caused by reversible airway obstruction.
Three factors contribute to bronchoconstriction – bronchial muscle contraction (triggered by a variety of stimuli), mucosal swelling or inflammation (mast cell and basophil degranulation causes inflammatory mediator release) and increased mucus production.
