Restrictive lung disease Flashcards
What two categories can restrictive lung disease be grouped into?
-Intrinsic lung disease
-Extrinsic disorders
What causes intrinsic lung disease giving an example.
Alterations to Lung parenchyma:
-Interstitial lung disease (ILD)
What are extrinsic lung disorders?
Anything that compresses lungs or limit expansion
What is the lung parenchyma?
The alveolar regions of the lung
Alveolar type 1 epithelial cell, What is it responsible for?
Gas exchange surface.
Alveolar type 2 epithelial cell, What is it responsible for?
Secrete surfactant to reduce surface tension
Contains stem cells for repair
What are fibroblasts responsible for in the lung parenchyma?
Produce extracellular matrix (ECM)
What are Alveolar macrophages responsible for in the lung parenchyma?
Phagocytose foreign material and acts as a surfactant
What is the interstitial space?
The space between alveolar epithelium and capillary endothelium
What will you find in clinical examination of someone with interstitial lung disease.
-Low oxygen saturations (resting or exertion)
-Fine bilateral inspiratory crackles
-Digital clubbing
What blood test investigations would you do for Interstitial lung disease?
-Anti-nuclear antibody (ANA),
-Rheumatoid factor (RhF),
-Anti-citrullinated peptide (CCP)
What imaging test would you do for Interstitial lung disease?
High resolution CT scan
What are the invasive tests that can be done for ILD?
Bronchoalveolar lavage (BAL)
Surgical lung biopsy (2-4% mortality)
What is the effect of scarring on the lungs in ILD?
Scarring makes the lung stiff which reduces compliance
What happens to lung volumes in ILD
(TLC, FRC, RV)
They decrease
What is FVC and what happens to it in ILD?
The amount of air that can be forcibly exhaled from your lungs after taking the deepest breath possible.
FVC decreases in Interstitial Lung Disease.
What happens to the FEV1/FVC ratio?
Normal or ↑ FEV1/ FVC ratio
What is idiopathic pulmonary fibrosis (IPF)?
Progressive, scarring lung disease of unknown cause.
What is idiopathic pulmonary fibrosis initiated by?
Alveolar epithelial injury
What are some characteristic features of IPF on a CT scan?
Is immunosuppression good or bad in Idiopathic pulmonary fibrosis.
It is harmful
What group of drugs can be given to slow disease progression in IPF? giving two examples
Antifibrotics:
-Nintedanib, tyrosine kinase inhibitor
-Pirfenidone, pyridine compound
What is hypersensitivity pneumonitis (HP)?
Interstitial Lung Disease caused by immune- mediated response in susceptible and sensitised individuals to inhaled environmental antigens.
What can hypersensitivity pneumonitis be classified into?
Acute HP
Chronic HP
