Restrictive Lung Disease Flashcards

1
Q

What defines a restrictive lung disease?

A

Decrease in lung volume - TLC reduction

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2
Q

What are lung parenchyma?

A

parts involved in gas transfer - respiratory bronchioles and alveoli

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3
Q

What is honeycombing?

A

In pulmonary fibrosis where there are clustered cystic air spaces, driven by cystic fibrosis

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4
Q

How can there be a reduction in lung volume?

A

lung parenchyma

pleura, chest wall or neuromuscular apparatus

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5
Q

What are the 2 major types of restrictive lung disease?

A

Intrinsic and extrinsic

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6
Q

What is the difference in pathology of intrinsic and extrinsic restrictive lung diseases?

A

Intrinsic - lung parenchyma inflamed and lung scarred

Extrinsic - chest wall, pleura and neuromuscular apparatus abnormalities

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7
Q

What are examples of diseases that cause intrinsic and extrinsic restrictive lung diseases?

A

Intrinsic - interstitial lung diseases

extrinsic - myasthenia gravis, obesity

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8
Q

What is the interstitium?

A

The tissue and space around the air sacs of the lungs

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9
Q

What is another name for interstitial lung disease?

A

parenchymal lung disease

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10
Q

What are the 2 main examples of interstitial lung disease and why?

A
Idiopathic interstitial pneumonia
Granulomatous DPLD (diffuse parenchymal lung disease) - sarcoidosis
HIGHEST INCIDENCE AND PREVALENCE
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11
Q

What are the classes of interstitial lung disease?

A

major
rare
unclassifiable

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12
Q

What are some risk factors for idiopathic pulmonary fibrosis?

A

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)

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13
Q

What are the symptoms of idiopathic pulmonary fibrosis?

A

clubbing
dry cough
exertional dyspnoea

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14
Q

What would be heard when listening to the lungs of someone with idiopathic pulmonary fibrosis?

A

Fine high pitched bibasilar inspiratory crackles

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15
Q

How is idiopathic pulmonary fibrosis diagnosed?

A
Chest HRCT (high resolution CT) - reticular changes, honeycombing, bronchiectasis
Surgical lung biopsy
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16
Q

What does management of idiopathic pulmonary fibrosis aim to do?

A

Improve symptoms and survival, preserve lung function and reduce adverse effects

17
Q

What are the 3 main medications given for idiopathic pulmonary fibrosis?

A

Pirfenidone
Nintedanib
Antacid therapy

18
Q

What is pirfenidone?

A

Anti-fibrotic agent
Increases progression free survival
Reduces physiological deterioration
AE = Gi, anorexia, skin rash, liver toxicity, photosensitivity

19
Q

What is Nintedanib?

A

Tyrosine kinase inhibitor
FVC decline
AE: weight loss, liver toxicity, diarrhoea

20
Q

What is antacid therapy?

A

Prevents gastro-oesophageal reflux which happens with IPF

AEs: infection, MI

21
Q

What are some non-pharmoacotherapy methods of management? When would you offer them?

A

Pulmonary rehab - to increase QoL, walking distance
Long term oxygen - hypoxemia at rest
Lung transplant - moderate severe

22
Q

What is sarcoidosis?

A

Multi-system granuloma
Immune system overdrive
acute, self limiting

23
Q

What does a restrictive lung disease look like on a volume flow loop?

A

Long and narrow

24
Q

What are corticosteroids?

A

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

25
Q

What is the cause of IDP and sarcoidosis?

A

unknown for both BUT
IDP: inflammation -> lung scarring & low oxygenation
sarcoidosis: inflammation -> multisystem granulomas