Clinical features and management of restrictive lung disease Flashcards
What is the physiological definition of restriction?
Forced vital capacity <80% of the predicted normal
Vital capacity - marker of restriction
What are causes of restriction?
“Interstitial” lung diseases
- Idiopathic pulmonary fibrosis
- Sarcoidosis
- Hypersensitivity pneumonitis
What are pleural causes of restriction?
- Pleural effusions
- Pneumothorax
- Pleural thickening
What are skeletal causes of restriction?
- Kyphoscoliosis
- Ankylosing spondylitis
- Thoracoplasty
- Rib fractures
Muscle causes of restriction
Amyotrophic lateral sclerosis
Sub-diaphragmatic causes of restriction
- Obesity
- Pregnancy
Where is the interstitium of the lungs?
it is a fluid-filled space between the epithelium of the alveolus and the endothelium of the capillary.
How can interstitial lung disease affect the interstitium?
- Interstitial lung disease can cause thickening of the interstitium.
- The thickening can be due to inflammation, scarring, or extra fluid (edema) etc.
What is idiopathic pulmonary fibrosis?
- A chronic, progressive form of fibrosis (scarring) of the interstitium.
What is sarcoidosis?
Multisystem granulomatous disease of unknown aetiology.
What is the histological hallmark of sarcoidosis?
Non-caseating granuloma
Who is more likely to get restrictive lung disease?
- Adults <40
- Women >Men
- World wide
How do you investigate restrictive lung disease?
- History and exam
- Chest X-ray
- Pulmonary function tests
- Bloods/ urinalysis/ ECG/ TB skin test/ eye exam
What would be seen on a chest X-ray?
- Hilar shadows are prominent because the glands are swollen up, bulge above the right hilum.
- Sometimes honeycomb lung
What is a further assessment that could be performed to investigate restriction?
Bronchoscopy including transbronchial biopsies and endobronchial ultrasound
