Interstitial Fibrosis
Cards (7)
- Idiopathic Pulmonary Fibrosis (IPF):
- IPF is a chronic relentlessly progressive fibrotic disorder of the lungs
- Unknown aetiology
- Typically affects adults greater than 40 yrs
- More common in males
- Average survival from time of diagnosis varies between 2.5 and 3.5 years depending on severity
- Some patients live for more than 10 years
- Pathophysiology of IPF:
- Characterized by abnormal and excessive deposition of fibrotic tissue in the pulmonary interstitium with minimal associated inflammation
- Clusters of fibroblasts form characteristic fibrogenic foci located in the injured lung
- Precise factors that initiate and maintain the fibrosis seen in IPF are unknown
- However risk factors include cigarette smoking, infections, environmental pollutants, chronic aspiration and drugs
- Clinical Signs & Symptoms:
- Symptoms are gradual in onset
- The most common:
- dyspnoea - patient feels breathless
- Non-productive cough
- Finger clubbing
- Inspiratory crackles on auscultation - produced by alveoli and airway supplying it opening up under inspiratory pressure
- Chest X-ray of IPF:
- white opacity across both lungs - thickening of the lung parenchyma and fibrotic change
- Spirometry:
- Lung function test results show restrictive defect - unable to take big enough breath in do to lung tissue being stiff
- decrease in Forced Vital Capacity
- Normal FEV1 (forced expiration volume in 1 second)
- increase in FEV1 / FVC ratio (of the total of the forced vital capacity, what % can you get out in the first second)
- Decrease in vital capacity
- Decrease in compliance associated with pulmonary fibrosis which leads to increased lung elastic recoil
- Treatment of IPF:
- Currently no consensus treatment IPF
- Treatment with drugs but minimal improvement
- Corticosteroids but no evidence base
- Immunosuppressive or cytotoxic agents
- Pulmonary rehabilitation
- Breathlessness management
- Oxygen therapy
- Lung transplantation
- IPF is a restrictive lung disease