Interstitial Lung Disease Flashcards
What is the interstitial space?
A potential space between alveolar cells and the capillary basement membrane, which is only apparent in disease states
What may the interstitial space contain in disease states?
Fibrous tissue, cells, or fluid
What is interstitial lung disease?
A group of diseases with a variety of causes, that all have similar pathological effects and clinical features
What is the pathophysiology of interstitial lung disease?
The development of fibrous tissue in the intersticium, producing a restrictive ventilatory defect
Why does the development of fibrous tissue in the intersticium produce a restrictive ventilatory defect?
Because it makes the lungs less compliant
Is airway resistance increased in interstitial lung disease?
No
How can the FEV1/FVC ratio be kept >70% in interstitial lung disease?
Due to increased radial traction on the airway keeping the airway open
What impairs gas exchange in interstitial lung disease?
Lengthening of the diffusion path
What is selectively affected in interstitial lung disease?
Oxygen uptake
Why is oxygen uptake selectively affected in interstitial lung disease?
As CO2 diffuses much more readily
What are the symptoms of interstitial lung disease?
- Shortness of breath
- Reduced exercise tolerance
- Dry cough
What are the signs of interstitial lung disease?
- Tachypnoea
- Tachycardia
- Reduced chest movement bilaterally
- Coarse crackles
What signs may be present in interstitial lung disease?
- Cyansosis
- Signs of right heart failure
When is clubbing seen in interstitial lung disease?
Cryptogenic fibrosing alveolitis
What are the categories of causes of interstitial lung disease?
- Occupational
- Treatment related
- Connective tissue disease
- Immunological
- Idiopathic
What are the occupational causes of interstitial lung disease?
- Abestosis
- Silicosis
- Coal workers pneumoconiosis
What are the treatment related causes of interstitial lung disease?
- Radiation
- Methotrexate
- Nitrofurantoin
- Amiodarone
- Chemotherapy
What connective tissue diseases can cause interstitial lung disease?
- Rheumatoid arthritis
- SLE
- Polymyositis
- Schleroderma
- Sjorgen’s
What are the immunological causes of interstitial lung disease?
- Sarcoidosis
- Hypersensitivity pneumonitis (EAA)
What are the idiopathic causes of interstitial lung disease?
- CFA/IPF
- UIP/NSIP
- DIP
- LIP
- RB-ILD
- COP (BOOP)
CFA- cryptogenic fibrosing alveolitis
IPF- idiopathic pulmonary fibrosis
UIP- usual interstitial pneumonia
NSIP- non-specific interstial pnuemonia
DIP- desquamative interstial pneumonia
LIP- lymphoid interstitial pnuemonia
RBILD- respiratory bronchiolitis interstiial lung disease
COP- crytogenic organising pneumonia
BOOP- bronchiolitis oblitirans organising pneuomnia
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What is fibrosing alveolitis?
A progressive inflammatory condition of an unknown cause
How common is fibrosing alveolitis?
Relatively rare, 3-5 cases per 100,000
How does the incidence of fibrosing alveolitis differ between the sexes?
It is two times more common in males
What are the histological features of fibrosing alveolitis?
Increased activated alveolar macrophages
What is the effect of increased activated alveolar macrophages in fibrosing alveolitis?
- Attract neutrophils and eosinophils
- Local lung damage
- Tissue destruction and fibrosis
Why do increased alveolar macrophages lead to local lung damage?
Due to ROS and proteases
What do patients report with fibrosing alveolitis?
Progressive shortness of breath on exercise, often with non-productive cough
Do patients with fibrosing alveolitis have finger clubbing?
Most do
What does the chest x-ray show in patients with fibrosing alveolitis?
Small lungs with micro-nodular shadowing predominating in the lower lobes, with ragged heart borders
When can fibrosing alveolitis be restrained?
In the early stages, less effective once fibrosis has developed
How can fibrosing alveolitis be restrained?
By treatment with high dose oral steroids
How is the effectiveness of treatment for fibrosing alveolitis monitored?
By repeated lung function tests
What causes extrinsic allergic alveolitis?
Inhalation of organic material triggers an allergic reaction in alveoli and bronchioles
Is extrinsic allergic alveolitis an acute or chronic condition?
Can be either
What are the characteristics of acute extrinsic allergic alveolitis?
- Sudden onset
- Rapidly progressing
Give an example of acute extrinsic allergic alveolitis
Farmer’s Lung
What is the antigen in Farmer’s Lung?
Thermophillic actinomycetes found in mouldy hay
How does Farmer’s Lung present?
- Influenza like illness 4-9 hours layer with a dry cough and breathlessness on exertion
- Fine mid and late inspiratory crackles
- May be a wheeze
Give an example of chronic extrinsic allergic alveolitis
Bird Fancier’s Lung
What causes Bird Fancier’s Lung?
Long term antigen exposure from pigeons and budgerigars
How does Bird Fancier’s lung present?
- Insidious malaise
- Dry cough and breathlessness over months and years
- Inspiratory crackles
What is meant by insidious malaise?
Feeling particularly unwell
Does finger clubbing occur with extrinsic allergic alveolitis?
No
What does a chest x-ray show in acute extrinsic allergic alveolitis?
Diffuse micro-nodular infiltrate, denser towards the hila
What does a chest x-ray show in chronic extrinsic allergic alveolitis?
May be almost normal, progressing to fibrosis in late disease
What do lung function tests show in extrinsic allergic alveolitis?
- Reduced compliance
- Reduced gas transfer
What causes asbestosis?
Inhalation of asbestos fibres
When does asbestosis often develop?
Long after the exposure
What is asbestos inhalation associated with?
- Three forms of disease
- Benign pleural plaques
- Asbestosis (pulmonary fibrosis)
- Mesothelioma
- Marked increase in lung cancer
What do asbestos fibres produce alveolitis?
When they can penetrate the alveoli
What happens once asbestos fibres have produced alveolitis?
- There is an influx of macrophages
- Alveolitis progresses to fibrosis
What does the influx of macrophages produce in alveolitis caused by asbestos?
Characteristic asbestosis bodies
What is required for a diagnosis of asbestosis?
A history of asbestos exposure
How does a patient with asbestosis present?
- Breathless on exertion
- Dry cough
- Inspiratory crackles at lung bases, which rise as the disease advances
What is the treatment for asbestosis?
None
What do lung function tests show in a patient with asbestosis?
- Small lungs
- Reduced compliance
- Impaired gas transfer
What causes sarcoidosis?
Unknown
What is sarcoidosis characterised by?
Non-caseating granulomas (non-necrotising) in multiple organs and body sites
Where is sarcoidosis most common?
In the lungs
What happens in sarcoidosis?
- Fluid is collected by lavage in the airways
- Alveoli contain lots of cells
What cells are present in large numbers in the alveoli in sarcoidosis?
Macrophages and lymphocytes
What is the epidemiology of sarcoidosis?
- More common in Afro-Caribbean and Asians than Caucasians
- Highest incidence in 30’s and 40’s
- More female cases
What can be determined from the fact that there is a difference in incidence between races?
There is a genetic predisposition