Pulmonary Fibrosis Flashcards
What is it?
A chronic condition that causes interstitial lung damage
What happens to the interstitial tissue?
It becomes fibrosed and loses its elasticity
What causes it? (3)
- Idiopathic
- Secondary to underlying disease e.g Infarction, TB, Pneumonia, Coal dust and Silica exposure
- Connective tissue diseases
Is it common?
It is the most common cause of interstitial lung damage, 7.5 per 100,000 people
Who does it affect?
Rare in young people, usual age of onset is 70 years old, often affects people with COPD
Risk factors (8)
Smoking, silica exposure, asbestos, mouldy foliage exposure, pigeon breeding, contaminated ventilation systems, chronic viral infections e.g HCV, GORD with microaspiration
Signs and Symptoms (10)
Dry cough, exertional dyspnoea, malaise, weight loss, arthralgia, cyanosis, clubbing, fine end inspiratory crackles, sleep apnoea, cor pulmonale
Differentials (5)
Heart failure, COPD, Lung cancer, Extrinsic allergic alveolitis, Asbestosis
What bloods would you do? (5)
FBC, U&E, LFT, CRP, Autoantibodies
Other Investigations (6)
Chest xray, Lung function testing, CT (shows honey combing), Bronchoalveolar lavage, Transbronchial biopsy, surgical lung biopsy
What is the treatment? (12)
Oxygen, Physiotherapy, regular exercise, weight control, flu and pneumococcus vaccines, smoking cessation, proton pump inhibitor therapy, opiates for excessive cough, PIRFENIDONE an anti-fibrotic, NINTEDANIB an intracellular Tyrosine Kinase Inhibitor with anti-fibrosis and anti-inflammatory, lung transplant, corticosteroids
Complications (5)
Pulmonary hypertension, lung cancer, P.E, right ventricular heart failure due to backlog, coronary heart disease
Is there a good prognosis?
Median uk survival is 2.5 years from diagnosis, 20% survive more than 5 years
