Restrictive lung diseases Flashcards
What is the other name for restrictive lung disease?
Interstitial lung disease
What is the interstitium?
Between the alveolar epithelium and capillary endothelium
What are the spirometry values in restrictive lung disease?
FVC<80%
FEV1/FVC= 90%
What is the acute response to interstitial lung injury?
Diffuse alveolar damage
What is the chronic response to interstitial lung injury?
Usual interstitial pneumonia
- Idiopathic pulmonary fibrosis
Granulomatous
- Sarcoidosis
- Hypersensitivity pneumonitis
What is diffuse alveolar damage (DAD)?
Acute lung condition with presence of hyalin membranes
What are the 2 stages of DAD?
Exudative stage:
- Pulmonary oedema
- Hyalin membrane formation
Proliferative stage:
- Interstitial inflammation
- Interstitial fibrosis
What are the histological features of DAD?
Protein rich oedema
Hyaline membranes
Fibrin
Denuded basement membrane
Epithelial proliferation
Fibroblast proliferation
Scarring
What is idiopathic pulmonary fibrosis (IPF)?
Chronic, progressive condition causing scar tissue (fibrosis) in the lungs
What is the pathophysiology of IPF?
- Damage to alveolar lining
- Fibroblasts proliferate into myofibroblasts
- Myofibroblasts secrete elastic and reticular fibres
- Myofibroblasts proliferate but no apoptosis
- Collagen accumulates thickening the interstitium
What does IPF look like on CT?
Honeycombing of the lungs
Thickening of the lungs
What is the treatment of IPF?
Supplemental oxygen
Anti-fibrotic
Lung transplant
What is sarcoidosis?
Immunological disorder which results in immune cells gathering and forming non-caseating granulomas
Results in small nodules throughout the body
What are the histological features of sarcoidosis?
Granulomas
No necrosis
Fibrosis
What are the symptoms of sarcoidosis?
Fever
Weight loss
Fatigue
Dyspnoea
Cough
Uveitis
Erythema nodosum
