Restrictive (interstitial) Lung Diseases Flashcards
What two parts is the interstitium between?
Alveolus and capillary
Why do you want the membranes involved in the interstitial space to be as close to each other as possible?
To facilitate the transport of CO2 (gas exchange)
In interstitial lung disease, what is the pathological process that is occurring?
The alveolar walls are becoming thickened
What does alveolar thickening lead to?
Reduced lung compliance (Stiffer lungs)
What happens to the FEV1/FVC ratio in ILD?
It remains normal
Why does the FEV1/FVC ratio remain normal during ILD?
As both FEV1 and FVC reduce?
What type of respiratory failure is linked to ILD?
Type 1
What is the definition of idiopathic?
Without known cause
What does the extensive damage to endothelial cells lead to in ‘diffuse alveolar damage?’
A huge outpouring of fluid and proteins
What is sarcoidosis?
A multisystem granulomatous disorder of unknown aetiology
What is special about the granulomas involved in sarcoidosis?
They are non-necrotising
What are the 5 main categories of antigens that cause hypersensitivity pneumonitis?
Thermophilic actinomycetes Bird/animal proteins Fungi Chemicals Others
What is hypersensitivity pneumonitis more likely than sarcoidosis to lead to?
Pulmonary fibrosis
Where does inflammation occur in hypersensitivity pneumonitis?
In the centriacinar region
What is the pathological process involved in idiopathic pulmonary fibrosis?
UIP (usual interstitial pneumonitis)
Where does the fibrosis generally take place in idiopathic pulmonary fibrosis?
Basal and posterior parts of the lung
What does gas flow through a membrane depend on?
Thickness and surface areas of the membrane
Gas pressure across the membrane
What is the physiological definition of restriction?
Forced vital capacity
What are the 3 main ‘interstitial lung diseases’?
Idiopathic pulmonary fibrosis
Sarcoidosis
Hypersensitivity pneumonitis
What are the 3 pleural causes of restrictive lung diseases?
Pleural effusions
Pneumothorax
Pleural thickening
What are the main skeletal causes of restrictive lung diseases?
Kyphoscoliosis
Ankylosing spondylitis
Thoracoplasty
Rib fractures
What are interstitial lung diseases?
Diseases that cause thickening of the interstitium and can result in pulmonary fibrosis
What is the remission rate of stage 3 sarcoidosis?
10-20%
What treatment would be given to someone presenting with sarcoidosis with skin lesions and a cough?
Topical steroids