Pulmonary fibrosis Flashcards
1
Q
What is the definition of pulmonary fibrosis?
A
Chronic interstitial lung damage and loss of elasticity of the lungs
2
Q
How common is pulmonary fibrosis?
A
Common, with incidence of around 7.44 per 100,000 population of the UK
3
Q
What is the pathology of pulmonary fibrosis?
A
- Remodelling of the interstitium/replacement of damaged lung tissue
- Chronic inflammation
- Hyperplasia of type II epithelial cells/type II pneumocytes
4
Q
What are the risk factors/aetiology of pulmonary fibrosis?
A
- Known cause:
- Occupational/environmental e.g. asbestos, silicosis, cotton workers lung
- Drugs e.g. nitrofurantoin, amiodarone, sulfasalazine
- Hypersensitivity reactions
- Infections e.g. TB, fungi, viral
- GORD
- Associated with systemic disorders:
- Sarcoidosis
- RA
- SLE, Sjorgren’s syndrome
- UC, renal tubular acidosis, autoimmune thyroid disease
- Idiopathic
- ↑ age
- Smoking
- COPD
5
Q
What are the signs/symptoms of pulmonary fibrosis?
A
- Dyspnoea on exertion
- Non productive paroxysmal cough
- Bilateral fine inspiratory crackles on auscultation
- Clubbing
- Abnormal CXR/CT
- Restrictive pulmonary spirometry – diffusing capacity of the lung for carbon monoxide
- Flu like symptoms, fatigue, weight loss
- Arthalgia, muscle pains, skin rashes
- Obstructive sleep apnoea
6
Q
What investigations are carried out for suspected pulmonary fibrosis?
A
- FBC, ESR, CRP, autoantibodies
- CXR
- Lung function test – spirometry
- High resolution CT of thorax
- Transbonchial/surgical lung biopsy
7
Q
What are the surgical treatments for pulmonary fibrosis?
A
Lung transplant
8
Q
What are the pharmacological treatments for pulmonary fibrosis?
A
- Oxygen
- Influenza and pneumococcal vaccinations
- PPI
- Opiates – in end stage
- Corticosteroids – acute exacerbation
- Pirfenidone – in FVC is between 50-80%
- Nitendanib
9
Q
What are the non pharmacological treatments for pulmonary fibrosis?
A
- Pulmonary rehabilitation
- Physiotherapy
- Regular exercise to control weight
- Smoking cessation
