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Respiratory System DO > Pathology of Restrictive Lung Disease - UIP > Flashcards

Pathology of Restrictive Lung Disease - UIP Flashcards

1
Q

What is UIP

A

Usual interstitial pneumonitis

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2
Q

In which diseases can UIP be seen

A 
Connective tissue diseases
Drug reaction
Post infection
Industrial exposure
Others
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3
Q

In what main types of connective tissue diseases can UIP be seen

A

Scleroderma and Rheumatoid disease

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4
Q

What type of industrial exposure can cause UIP

A

Asbestos

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5
Q

Why can UIP be called IPF or CFA, What do they stand for

A

Most cases of UIP are cryptogenic or idiopathic
Iiopathic pulmonary fibrosis (IPF)
Cryptogenic fibrosing alveolitis (CFA)

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6
Q

What will the histopathology of UIP show

A

Patchy interstitial chronic inflammation
Type II pneumocyte hyperplasia
Smooth muscle and vascular proliferation

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7
Q

Give 6 common features of UIP

A

Occurs in the elderly (over 50)
Males affected more than females
Progressive disease – many die within 5 years of presentation
CXR shows Basal/Posterior, Diffuse infiltrates, Cysts, ‘Ground Glass’
Restrictive PFT & Reduced Gas Transfer
Poor Prognosis

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8
Q

What are the symptoms of UIP

A 
Dyspnoea
Cough
Basal Crackles
Cyanosis
Clubbing
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9
Q

Which agents have been effective in UIP and which are ineffective

A

Effective - anti-angiogenic agents

Ineffective - Steriods

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10
Q

In what two forms can the air in the airways move and what is it dependent on

A

Laminar or Turbulent

Dependent on the pressure difference

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11
Q

How is gas exchanged beyond the terminal bronchiole

A

Diffusion

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12
Q

What does the affinity of Hb for oxygen allow

A

The blood which leaves the capillary bed is 98% saturated for FIO2 of only 0.21

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13
Q

What does the soulbility of CO2 allow

A

CO2 can rapidly equilibrate between the blood and air

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14
Q

What are normal PaO2 levels

A

10.5 - 13.5 kPa

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15
Q

What are normal PaCO2 levels

A

4.8-6.0 kPa

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16
Q

What are PaO2 levels in type I respiratory failure

A

PaO2 <8 kPa (PaCO2 normal or low)

17
Q

What are PaCO2 levels in type I respiratory failure

A

PaCO2 >6.5 kPa (PaO2 usually low)

18
Q

What are the 4 abnormal states associated with hypoxaemia

A

Alveolar Hypoventilation
Shunt
Ventilation / Perfusion imbalance - V/Q
Diffusion impairment

19
Q

What is FIO2

A

The Fraction of Inspired air which is Oxygen

20
Q

What is alveolar hypoventilation

A

The amount of air moved in and out of the lungs

21
Q

What happens in alveolar hypoventilation

A

PACO2 increases so PaCO2 increases. The increase in PACO2 causes a decrease in PAO2 causing PaO2 to fall

22
Q

How is a fall in PaO2 due to hypoventilation corrected

A

By raising FIO2

23
Q

What is the normal V/Q level

A

We normally breath 4L/min and CO is 5L/min so normal V/Q is 4/5 or 0.8

24
Q

What is the commonest cause of hypoxaemia encountered clinically

A

Low V/Q

25
Q

How can low V/Q in some alveoli arise

A

Due to local alveolar hypoventilation due to some focal disease

26
Q

What does hypoxaemia due to low V/Q respond to

A

Small increases in FIO2

27
Q

What is diffusion impairment

A

When it takes longer for blood and alveolar air to equilibrate, particularly for oxygen.

28
Q

What is the gas flow through a membrane dependent on

A

The thickness and surface area of the membrane and the gas pressure across it.

29
Q

Why can CO2 diffuse 20 times faster than O2

A

Due to its greater solubility

30
Q

Do diseases which impair gas diffusion change CO2 levels

A

Normally no

31
Q

What does diffusion impairment mean

A

That it takes longer for blood and alveolar air to equilibrate, particularly for oxygen.

32
Q

How long does equilibration normally take

A

0.25 seconds

33
Q

What is the normal capillary transit time

A

0.75 seconds

34
Q

How long can equilibration take in disease

A

May take close to 0.75 seconds so PaO2 is maintained at rest however there can be serious falls in PaO2 on exercise as RBC are spending less time in the capillary bed

35
Q

How can hypoxaemia be corrected in diffusion impairment

A

Increasing FIO2

This increases PAO2, thus increasing rate of diffusion

36
Q

What is a shunt

A

When blood passes from the right side to the left side of the heart without contacting ventilated alveoli

37
Q

What is a normal shunt size

A

2-4%

38
Q

What can cause pathological shunts

A

AV malformations
Congenital heart disease
Pulmonary disease

39
Q

How will a large shunt respond to increases in FIO2

A

Poorly

The blood leaving the normal lung is already 98% saturated

Respiratory System DO (33 decks)

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