Respiratory Flashcards
What is pulmonary fibrosis?
Group of diseases which lead to interstitial lung damage and ultimately fibrosis
What are causes of pulmonary fibrosis?
1) Lung damage
2) Irritants
3) Diffuse parenchymal lung disease
4) CT disease
5) Medications
6) Hypersensitivity pneumonitis
What causes of lung damage can cause pulmonary fibrosis?
Infarction, pneumonia, TB
What irritants can cause pulmonary fibrosis?
Coal dust, silica
What diffuse parenchymal lung diseases can cause pulmonary fibrosis?
Idiopathic pulmonary fibrosis, extrinsic allergic alveolitis
Which CT diseases can cause pulmonary fibrosis?
RA, SLE, systemic sclerosis, Sjögren’s syndrome
Which medications can cause pulmonary fibrosis?
Amiodarone, nitrofurantoin, bleomycin
What are causes of hypersensitivity pneumonitis that can cause pulmonary fibrosis?
Exposure to birds or moulds
What is idiopathic pulmonary fibrosis?
Progressive, fibrotic lung disease of unknown cause. gradual onset
What is the typical patient with idiopathic pulmonary fibrosis?
70 years old, male, smoker
What are the symptoms of idiopathic pulmonary fibrosis?
1) Dry cough
2) SOB
3) Fatigue
4) Arthralgia
What are the signs of idiopathic pulmonary fibrosis?
1) Cyanosis
2) Clubbing
3) Fine end-inspiratory crackles (most prominent at bases bilaterally but can be heard throughout
4) Reduced inspiratory phase
What are complications of idiopathic pulmonary fibrosis?
1) Type 2 respiratory failure
2) Increased risk of lung cancer
3) Cor pulmonale
4) 50% mortality in 5 years
What investigations do you do in idiopathic pulmonary fibrosis?
1) Blood tests - CRP, ANA, Rf
2) ABG - hypoxia and hypercapnia are likely
3) CXR - bilateral lower zone reticulo-nodular shadowing
4) High resolution CT chest (to categorise extent of disease) - reticulonodular shadowing and honeycomb lung
5) Spirometry (to assess for restrictive and obstructive pulmonary deficit) - restrictive deficit with reduced transfer factor
6) Rule out malignancy and other conditions
What are management options for idiopathic pulmonary fibrosis?
1) Conservative - stop smoking, pulmonary rehabilitation
2) Medical - long term oxygen therapy, antifibrotic drugs e.g. pirfenidone, symptom control in right sided HF
3) Surgical - lung transplant is the only cure
