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Cardiovascular & Respiratory Medicine > Restrictive lung disease > Flashcards

Restrictive lung disease Flashcards

1
Q

What two categories can restrictive lung disease be grouped into?

A

-Intrinsic lung disease
-Extrinsic disorders

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2
Q

What causes intrinsic lung disease giving an example.

A

Alterations to Lung parenchyma:
-Interstitial lung disease (ILD)

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3
Q

What are extrinsic lung disorders?

A

Anything that compresses lungs or limit expansion

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4
Q

What is the lung parenchyma?

A

The alveolar regions of the lung

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5
Q

Alveolar type 1 epithelial cell, What is it responsible for?

A

Gas exchange surface.

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6
Q

Alveolar type 2 epithelial cell, What is it responsible for?

A

Secrete surfactant to reduce surface tension
Contains stem cells for repair

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7
Q

What are fibroblasts responsible for in the lung parenchyma?

A

Produce extracellular matrix (ECM)

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8
Q

What are Alveolar macrophages responsible for in the lung parenchyma?

A

Phagocytose foreign material and acts as a surfactant

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9
Q

What is the interstitial space?

A

The space between alveolar epithelium and capillary endothelium

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10
Q

What will you find in clinical examination of someone with interstitial lung disease.

A

-Low oxygen saturations (resting or exertion)

-Fine bilateral inspiratory crackles

-Digital clubbing

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11
Q

What blood test investigations would you do for Interstitial lung disease?

A

-Anti-nuclear antibody (ANA),
-Rheumatoid factor (RhF),
-Anti-citrullinated peptide (CCP)

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12
Q

What imaging test would you do for Interstitial lung disease?

A

High resolution CT scan

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13
Q

What are the invasive tests that can be done for ILD?

A

Bronchoalveolar lavage (BAL)

Surgical lung biopsy (2-4% mortality)

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14
Q

What is the effect of scarring on the lungs in ILD?

A

Scarring makes the lung stiff which reduces compliance

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15
Q

What happens to lung volumes in ILD
(TLC, FRC, RV)

A

They decrease

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16
Q

What is FVC and what happens to it in ILD?

A

The amount of air that can be forcibly exhaled from your lungs after taking the deepest breath possible.
FVC decreases in Interstitial Lung Disease.

17
Q

What happens to the FEV1/FVC ratio?

A

Normal or ↑ FEV1/ FVC ratio

18
Q

What is idiopathic pulmonary fibrosis (IPF)?

A

Progressive, scarring lung disease of unknown cause.

19
Q

What is idiopathic pulmonary fibrosis initiated by?

A

Alveolar epithelial injury

20
Q

What are some characteristic features of IPF on a CT scan?

A
21
Q

Is immunosuppression good or bad in Idiopathic pulmonary fibrosis.

A

It is harmful

22
Q

What group of drugs can be given to slow disease progression in IPF? giving two examples

A

Antifibrotics:
-Nintedanib, tyrosine kinase inhibitor
-Pirfenidone, pyridine compound

23
Q

What is hypersensitivity pneumonitis (HP)?

A

Interstitial Lung Disease caused by immune- mediated response in susceptible and sensitised individuals to inhaled environmental antigens.

24
Q

What can hypersensitivity pneumonitis be classified into?

A

Acute HP
Chronic HP

25
Q

How would you diagnose HP?

A

-Inspiratory ‘squeaks’ on auscultation caused by co-existing bronchiolitis
-HRCT
-Bronchoalveolar lavage, lymphocyte count >30%
-IgG antibodies to potential antigens

26
Q

How do you treat Hypersensitivity pneumonitis?

A

-Complete antigen removal/ avoidance
-Corticosteroids
-Immunosuppressants & anti fibrotics e.g Nintedanib

27
Q

What is systemic sclerosis associated ILD?

A

Autoimmune connective tissue disease characterised by immune dysregulation and progressive fibrosis that affects skin, with variable internal organ involvement

28
Q

What is classification of systemic sclerosis associated ILD based on?

A

Level of skin involvement.

29
Q

What is more common if there is limited cutaneous involvment SSc

A

Pulmonary hypertension

30
Q

What is more common with diffuse
(large) cutaneous involvement SSc?

A

Interstitial lung disease more common

31
Q

Which IL do plasma cells release?

A

IL 6

32
Q

What do these HRCT images show?

A

Non-specific interstitial pneumonia in SSc-ILD

33
Q

What is the most common type of ILD?

A

IPF, idiopathic pulmonary fibrosis.

Cardiovascular & Respiratory Medicine (12 decks)

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