Interstitial Lung Disease Flashcards
What is interstitial space?
- A potential space between alveolar cells and the capillary basement membrane which is only apparent in disease states, where it may contain fibrous tissue, cells or fluid
What is interstitial lung disease?
- Group of diseases with a variety of cuases that have similar pathophysiological effects and clinical features
What is the pathophysiology of interstitial lung disease?
- Fibrous tissue develops in the interstitium, making lungs less compliant (restrictive defect)
- Diffusion path between alveolar air and blood impairs gas exchange. O2 uptake affected selectively
What are the clinical features of interstitial lung disease?
Shortness of breath
Reduced exercise tolerance
Dry cough
Give four signs of Interstitial Lung Disease
- Tachypnoea
- Tachycardia
- Reduced chest movement
- Coarse crackles
Give five causes of interstitial lung disease?
- Occupational
- Treatment related
- Connective tissue disease • Immunological • Idiopathic
Give three occupational causes of interstitial lung disease
- Asbestosis
- Silicosis
- Pneumoconiosis
Give three treatment related causes of Interstitial Lung Disease
- Radiation
- Methotrexate
- Nitrofurantoin
Give three connective tissue disease
• Rh. Arthritis • Polymyositis • Scleroderma
Give two immunological causes of Interstitial Lung Disease
• Sarcoidosis • Hypersensitivity pneumonitis
What is fibrosing alveolitis?
- Progressive inflammatory condition of unknown cause
- Increased activated alveolar macrophages
- Attract neutrophils and eosinophils
- Local lung damage due to ROS and proteases
- Tissue destruction and fibrosis
What do patients present with when they have fibrosing alveolitis?
- Progressive shortness of breath on exercise, often combined with a productive cough
- Finger clubbing
What does the chest x ray of someone with fibrosing alveolitis show?
Small lungs with micro-nodular shadowing predominating in the lower lobes, with ragged heart borders
How is fibrosing alveolitis treated, and what are the limitations of this treatment?
Treated with high dose oral steroids in the early stages
Less effective once fibrosis has developed
How is treatment of fibrosing alveolitis monitored?
• By repeated lung function tests
What is extrinsic allergic alveolitis?
• Inhalation of organic material which triggers an allergic reaction in alveoli and bronchioles
What are two states of the condition?
• Can be acute or chronic
Outline acute extrinsic allergic alveolits
Farmers lung
- Thermophilic actinomycetes found in mouldy hay
- Inhalation causes influenza like illness 4-9 hours later with a dry cough and dyspnoea on exertion.
- Fine mid and late inspiratory crackles
- Wheeze
Outline chronic extrinsic allergic alveolitis
- Long term antigen exposure = Faeces of pidgeons/budgies
- Insidious malaise
- Dry cough and breathlessness over months and years
- Inspiratory crackles
What sign does not occur in any types of allergic alveolitis?
• Finger clubbing
What does the x-ray show in acute extrinsic allergic alveolitis?
Diffuse micro-nodular infiltrate denser towards the hila
What does the x-ray show in chronis extrinsic allergic alveolitis?
Normal, may progress to fibrosis
What will a lung function test show in extrinsic allergic alveolitis?
Lung function test will show reduced compliance and reduced gas transfer
What is asbestosis?
- Inhalation of asbestos fibres causes a disease to develop long after exposure
Give three different types of disease as a result of asbestos inhalation
- Benign pleural plaques
- Asbestosis
- Mesothelioma
What do asbestos fibres do in the lungs?
- Penetrate to the alveoli, causing alveolitis
- Causes an influx of macrophages which produce distinctive asbestos bodies
- Alveolitis progresses to fibrosis
What are the symptoms of asbestos related disease?
- Patient breathless on exertion and a dry cough
- Inspiratory crackles at the lung base, which rise as the disease advance
What do lung function tests of someone with asbestos related lung disease show?
• Small lungs, reduced compliance and impaired gas transfer