Restrictive Lung Disease Flashcards
What defines a restrictive lung disease?
Decrease in lung volume - TLC reduction
What are lung parenchyma?
parts involved in gas transfer - respiratory bronchioles and alveoli
What is honeycombing?
In pulmonary fibrosis where there are clustered cystic air spaces, driven by cystic fibrosis
How can there be a reduction in lung volume?
lung parenchyma
pleura, chest wall or neuromuscular apparatus
What are the 2 major types of restrictive lung disease?
Intrinsic and extrinsic
What is the difference in pathology of intrinsic and extrinsic restrictive lung diseases?
Intrinsic - lung parenchyma inflamed and lung scarred
Extrinsic - chest wall, pleura and neuromuscular apparatus abnormalities
What are examples of diseases that cause intrinsic and extrinsic restrictive lung diseases?
Intrinsic - interstitial lung diseases
extrinsic - myasthenia gravis, obesity
What is the interstitium?
The tissue and space around the air sacs of the lungs
What is another name for interstitial lung disease?
parenchymal lung disease
What are the 2 main examples of interstitial lung disease and why?
Idiopathic interstitial pneumonia Granulomatous DPLD (diffuse parenchymal lung disease) - sarcoidosis HIGHEST INCIDENCE AND PREVALENCE
What are the classes of interstitial lung disease?
major
rare
unclassifiable
What are some risk factors for idiopathic pulmonary fibrosis?
Smoking, environmental exposures, chronic viral infections, abnormal acid reflux, family history (as all lead to airway remodelling therefore impaired oxygenation, all progressive and incurable damage)
What are the symptoms of idiopathic pulmonary fibrosis?
clubbing
dry cough
exertional dyspnoea
What would be heard when listening to the lungs of someone with idiopathic pulmonary fibrosis?
Fine high pitched bibasilar inspiratory crackles
How is idiopathic pulmonary fibrosis diagnosed?
Chest HRCT (high resolution CT) - reticular changes, honeycombing, bronchiectasis Surgical lung biopsy
What does management of idiopathic pulmonary fibrosis aim to do?
Improve symptoms and survival, preserve lung function and reduce adverse effects
What are the 3 main medications given for idiopathic pulmonary fibrosis?
Pirfenidone
Nintedanib
Antacid therapy
What is pirfenidone?
Anti-fibrotic agent
Increases progression free survival
Reduces physiological deterioration
AE = Gi, anorexia, skin rash, liver toxicity, photosensitivity
What is Nintedanib?
Tyrosine kinase inhibitor
FVC decline
AE: weight loss, liver toxicity, diarrhoea
What is antacid therapy?
Prevents gastro-oesophageal reflux which happens with IPF
AEs: infection, MI
What are some non-pharmoacotherapy methods of management? When would you offer them?
Pulmonary rehab - to increase QoL, walking distance
Long term oxygen - hypoxemia at rest
Lung transplant - moderate severe
What is sarcoidosis?
Multi-system granuloma
Immune system overdrive
acute, self limiting
What does a restrictive lung disease look like on a volume flow loop?
Long and narrow
What are corticosteroids?
broad spectrum anti-inflammatory drugs
diffuse across membrane as lipid loving
site of action is nucleus
binds to glucocorticoid receptors leading to upregulating anti-inflammatory genes
