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Idiopathic Pulmonary Fibrosis (Symptoms (Malaise, Weight loss, Exertional…
Idiopathic Pulmonary Fibrosis
Definition
A type of idiopathic interstitial pneumonia
Inflammatory cell infiltrate and pulmonary fibrosis of unknown cause
Epidemiology
50% 5yr survival rate
Presents in late 60s
Symptoms
Malaise
Weight loss
Exertional dyspnoea
Arthralgia
Dry cough
Signs
Finger clubbing
Fine end-inspiratory crackles
Cyanosis
Complications
Increased risk of lung cancer
Respiratory failure
Investigations
Chest X-ray: Decreased lung volume, bilateral lower zone reticulonodular shadows, honeycomb lung
Spirometry: restrictive
ABG: low PaO2, high PaCO2
Bloods: raised CRP, raised Igs, ANA/RF factor
Lung biopsy: shows usual interstitial pneumonia
Treatment
Pulmonary rehabilitation
Opiates
Oxygen
Best supportive care + palliation