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Respiratory Module > Lecture 6 - Idiopathic Pulmonary Fibrosis > Flashcards

Lecture 6 - Idiopathic Pulmonary Fibrosis Flashcards

1
Q

What is the most common interstitial lung diseases?

A

Idiopathic pulmonary fibrosis

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2
Q

How many interstitial lung diseases are there?

A

200

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3
Q

What is rheumatology?

A

Inflammation in bones and joints

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4
Q

What is asbestos?

A

Mediated by dust and fits into the overall family of interstitial lung diseases

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5
Q

What does idiopathic mean?

A

Means you don’t know the cause

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6
Q

What is hypersensitive pneumonia?

A

an immune response to the enviornment -allergic reaction e.g. pigeon feathers or plants

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7
Q

When does asbestos turn unsafe?

A

It is safe in the ground but if you mine it the fibres cab get into the lungs, when the fibres break they break into the perfect size to be repaired into the deep alveoli in the lungs (5 microns)

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8
Q

What can asbestos cause in the lungs?

A

Cause many different diseases, leads to pleural thickening, calcification on the X rays and calyces. Asbestos can cause cancers

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9
Q

Is IPF male specific?

A

To is a disease of the elderly and mainly male specific

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10
Q

What is different to the phenotype of IPF and asbestos related disease?

A

IPF can actually have a similar phenotype as asbestos related diseases, it has the same X-ray changes, it can have the same changes of lung function. However you know what causes asbestos but you don’t know what cause IPF

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11
Q

What is the interstitium?

A

Is the tissue between the cells of the alveoli and circulation

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12
Q

What do the blue fibres indicate in the interstitium?

A

Indicates the fact you get a thickening of the interstitium

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13
Q

What do you need for an efficient gas diffusion?

A

You need a thin membrane

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14
Q

What happens to the interstitium during fibrosis (gas exchange)?

A

The gap for diffusion of oxygen is going to increase so the ability to diffuse oxygen is going to go down and will the diffusion capacity. Gas exchange is impaired by the interstitial lung disease.

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15
Q

What happens to the lungs in fibrosis?

A

The lungs become smaller, really difficult to cut up with scalpel as it has a thought fibrotic material in it. Small lung decreases lung function

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16
Q

What does too much collagen in the lung cause?

A

Fibrosis

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17
Q

What do fibroblasts cause?

A

Cause chemical fibrosis and release collagen, a clump of fibroblasts secrete lots more collagen then you need

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18
Q

What is pathophysiology?

A

It is a scarring process - an aberrant normal healing process

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19
Q

How many people live with IPF?

A

Over 30,000

20
Q

What is the survival rate of IPF?

A

3-5 years, it is an orphan lung disease as not enough is known about it

21
Q

What is gastrooesphagus reflex?

A

It is a factor to maybe cause IPF

22
Q

What is the subclincial incubation period?

A

Where people aren’t getting any loss of function, but gradually gets worse over time

23
Q

What is the FEV1 like in IPF?

A

It is abnormal

24
Q

What does pirfenidone do?

A

Suppresses fibroblast proliferation via the TFG-beta inhibition

25
Q

What are the side effects for pirfenidone?

A

GI upset, weight loss, photosensitive rash and hepatotoxicity

26
Q

What is hepatoxcity?

A

Poisoning of the liver

27
Q

Does Pirfenudone work for everybody?

A

No it doesn’t work for everybody, but it better than IPF

28
Q

What is Nintedanib?

A

It is a triple TKI (tyrosine protein kinase inhibitor)

29
Q

What are the side effects for Nintedanib?

A

GI upset, diarrhoea, hepatotoxcity

30
Q

What are the cautions for taking Nintedanib?

A

If if you are taking anticoagulants, have cardiac disease or liver disease

31
Q

What are the main anti-fibrotic therapies?

A

Pirfenidone and Nintedanib - they slow the rate of decline of lung function

32
Q

Describe pirfenidone

A

Take 9 capsules each day, slows down inflammation and the build-up of scar tissue in the lungs. The drug slowed down the loss of lung function in most people with IPF, decreased symptoms decline and also improved life expectancy

33
Q

What is the daily cost of pirfenidone?

A

Daily cost of £71.70 - it is only recommended if the person has a FVC of 50%-80% predicted

34
Q

Is there a cure for IPF?

A

NO

35
Q

What is a bad candidate for a lung transplant?

A

If the patient is too old or unfit

36
Q

Can you inspire and swallow at the same time?

A

NO

37
Q

Describe the overall respiratory control system (negative feedback)

A

Central controller - effectors (respiratory muscles) - sensors (chemoreceptors, lungs )

38
Q

What do people carry with them if they have IPF?

A

An oxygen tank - they can have as much oxygen as they need

39
Q

What risks are considered to be higher for IPF?

A

If you smoke or have family history of IPF, this increases with age

40
Q

What is the most common symptom of IPF?

A

Shortness of breath and cough. Some people may not have symptoms at risk but some symptoms can develop and get worse as the disease progresses

41
Q

Does the IPF process vary in different people?

A

IPF can be different from person to person, scarring may happen slowly or quickly. In some people the disease stays for the same years. In others the condition gets worse more quickly

42
Q

What are some other complications from IPF?

A

Pulmonary hypertension and respiratory failure which happen when the lungs cannot deliver enough oxygen into the bloodstream without support. This prevents the brains and other organs from getting the oxygen they need

43
Q

When a doctor does a physical exam for IPF what do they see?

A

Blue hands and feet from not enough oxygen in the blood. Clubbing of the fingers or toes and high-pitched crackles when listening to the lungs

44
Q

What is clubbing of the fingers and toes?

A

Softening of the nail bed, changing of the angle of the nail, bulging of the lat part of the finger and downward curvation of the nail

45
Q

What diagnostics tests and procedures can be used to test for IPF?

A

Hugh resolution chest CT scan, lung biopsy and chest X-ray

Respiratory Module (10 decks)

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