Bronchiectasis, IPF, sarcoidosis Flashcards
Pathophysiology of bronchiectasis
- The irreversible airway dilation seen in bronchiectasis occurs as a result of chronic inflammation caused by immune response to infection.
- The dilation creates a vicious cycle in which the airway is more susceptible to repeat colonisation, causing recurrent episodes.
Main organisms associated with bronchiectasis
- Haemophilus influenzae
- Streptococcus pneumoniae
- Staphylococcus aureus
- Pseudomonas aeurginosa
Symptoms and signs of bronchiectasis
- Symptoms
- Persistent cough
- Copious purulent sputum
- Intermittent haemoptysis
- Dyspnoea
- Signs
- Finger clubbing
- Coarse inspiratory crepitations
- Wheeze
Complications of bronchiectasis
- Pneumonia
- Pleural effusion
- Pneumothorax
- Haemoptysis
- Cerebral abscess
- Amyloidosis
Investigations for bronchiectasis
- CXR - may show tram tracks/fluid levels
- High resolution CT scan - gold standard, helps diagnose the condition and assess severity
- Spirometry
- Obtain a sputum sample for culture and microscopy
- Other investigations:
- Bloods: FBC, U&Es, serum immunoglobulins, Aspergillus antigen test
- Assessment of genetic disease if indicated
- Bronchoscopy may be indicated
Management of bronchiectasis
- Airway clearance - taught by chest physiotherapist
- Assess response to beta-2 agonists and anticholinergic bronchodilator therapy
- Start empirical antibiotic therapy (which specifically includes inhaled antibiotics) while awaiting sputum microbiology - amoxicillin or clarithromycin
- If poor response consider lung surgery - lung resection surgery or lung transplant surgery
Treatment of ABPA
- Oral corticosteroids (prednisolone) and itraconazole
- Chest physiotherapy
- Bronchodilators
Interstitial lung disease aetiology
- The most common presentation is idiopathic pulmonary fibrosis
- ILD can also occur as a result of medication use, autoimmune disease such as sarcoidosis or lupus, or occupational exposure to inhaled substances
Clinical features of idiopathic pulmonary fibrosis
- Progressive dyspnoea that is worse on exertion
- Dry cough
- Finger clubbing
- Bilateral inspiratory crackles on auscultation
- PFTs demonstrate a restrictive type pattern in IPF
Complications of idiopathic pulmonary fibrosis
- Respiratory failure
- Increased risk of lung cancer
Investigations for idiopathic pulmonary fibrosis
- Conduct an adequate history and physical examination
- Perform blood tests - ABG, FBC
- PFTs and gas trasfer (DLCO reduced in IPF)
- spirometry shows restrictive pattern (FEV1 normal/decreased, with FEV1/FVC increased)
- CXR
- will show bilateral lower zone reticulonodular shadows
- Perform high resolution CT of the thorax
- may demonstrate ground glass appearance
Management of idiopathic pulmonary fibrosis
Supportive care: oxygen, pulmonary rehabilitation, opiates, palliative care input.
All patients should be considered for current clinical trials or lung transplantation. It is strongly recommended that high-dose steroids are not used except where the diagnosis of IPF is in doubt.
Idiopathic pulmonary fibrosis prognosis
Average life expectancy ranging from 3 to 5 years from diagnosis
Define sarcoidosis
Sarcoidosis refers to a chronic granulomatous disorder of unknown cause affecting multiple organ systems and is characterised histologically by non-caseating granulomas.
Epidemiology of sarcoidosis
- Worldwide prevalence of 6 per 100,000
- Higher incidence of Afro-Caribbean and Scandinavian populations
- Traditionally affects people in their second to fourth decade