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1st Year Systems > Restrictive (Interstitial) Lung Diseases > Flashcards

Restrictive (Interstitial) Lung Diseases Flashcards

1
Q

What is interstitium?

A

The connective tissue space

around the airways and vessels

and the space between the basement membranes of the alveolar walls

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2
Q

Describe the relationship between the normal alveolar wall and most of the alveolar epithelial (pneumocyte) & interstitial capillary endothelial cell basement membranes

A

They are in direct contact

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3
Q

What is the material which impedes the elasticity of the alveoli (reducing compliance)?

A

Collagen

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4
Q

What are the characteristics of Restrictive – Diffuse - Interstitial Lung Disease?

A

•Reduced Lung Compliance

  • Stiff Lungs

•Low FEV1 & Low FVC but FEV1/FVC normal ratio

•

•Reduced Gas Transfer (Tco or Kco)

-Diffusion abnormality

•Ventilation/Perfusion Imbalance

When small airways affected by pathology

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5
Q

Is there airflow limitation in restrictive lung disease?

A

NO

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6
Q

What are the clinical presentations for restrictive lung disease?

A
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7
Q

Which one is Normal? Emphysema? Interstitial Lung Disease?

A
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8
Q

What are the outcomes of Parenchymal (Interstitial) Lung Injury?

A

Acute inflammation (can develop into chronic response in rare circumstances) or Chronic inflammation

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9
Q

What are the three outcomes of Chronic response?

A
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10
Q

Which Chronic restrictive disease is most likely to develop end - stage honeycomb lung?

A

Usual Interstitial Pneumonitis

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11
Q

What does DAD stand for?

A

Difuse alveolar damage

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12
Q

What is DAD asociated with?

A
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13
Q

What are the exudative stages of DADS?

A

Edema and Hyaline membranes

Edema - Vessels become massively leaky - moreso than in a normal response

Hyaline membranes - lots of proteins

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14
Q

What are the proliferative stages of DADS?

A
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15
Q

What are the histological features of DADS?

A
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16
Q

What is the commonest interstitial lung disease that you will encounter?

A

Sarcoidosis

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17
Q

Apart from sarcoidosis what is the other granulomatous respnse?

A

Hypersensitivity pneumonitis

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18
Q

What is the histopathology of sarcoidosis?

A
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19
Q

Why is sarcoidosis called multisystem?

A

Involves many organs

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20
Q

Do most patients with sarcoidosis reach end stage fibrotic lung disease?

A

No ???

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21
Q

What is the presentation of sarcoidosis?

A
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22
Q

What is the diagnosis of sarcoidosis?

A
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23
Q

What is ACE?

A

A converting enzyme, converts angiotensin 1 to angiotensin 2.

Angiotensin II helps increase blood pressure by causing small blood vessels in the body to tighten or narrow.

Doctors most often use the ACE level test to monitor a disease called sarcoidosis. This condition causes inflammatory cells called granulomas to form in the body, leading to organ inflammation.

The granulomas associated with sarcoidosis increase the amount of ACE in the blood.

24
Q

What are the hypersensitivity pneumonitis antiges?

A
25
Q

What is the accute presentation for Hypersensitivity Pneumonitis?

A
26
Q

What is the chronic presentation of Hypersensitivity pneumonitis?

A
27
Q

What type of hypersensitivity causes Hypersensitivity Pneumonitis?

A

•Immune complex mediated combined Type III and Type IV Hypersensitivity reaction

28
Q

Describe the granulomata in Hypersensitivity Pneumonitits

A

•Soft centriacinar epithelioid granulomata, causing interstitial pneumonitis

29
Q

Hypersensitivty pneumonitis is said to cause Bronchiolitis obliterans, what does this mean?

A

Inflammatory obstruction of bronchioles, these bronchioles become damaged and ingflamed by chemical particles or respiratory infections. Features extensive scarring that blocks airways.

30
Q

What are the causes of Usual interstitial pneumonitis?

A

Connective tissue diseases - •scleroderma and rheumatoid disease

Drugs

Asbestos

Viruses

Idiopathic/cryptogenic reasons

31
Q

What is cryptogenic fibrosing alveolitis?

A

Old fashioned name for UIP

32
Q

Describe the interstitium of UIP?

A

Patchy chronic inflammatino

Type 2 pneumocytes hyperplasia

Smooth muscle and vascular proliferation

Proliferating fibroblastic foci

33
Q

What are Proliferating Fibroblastic Foci?

A

Nodules of fibrous tissue in the walls of the alveoli, reflect the severity of the disease

34
Q

Who usually suffers from Idiopathic Pulmonary Fibrosis?

A
35
Q

What is the pathology of Idiopathic Pulmonary Fibrosis?

A

UIP

36
Q

What is the clinical presentation of Idiopathic Pulmonary Fibrosis?

A
37
Q

What is the prognosis for Idiopathic Pulmonary Fibrosis?

A

Usually dead within 5 years

38
Q

What will you see in the ches X-ray for someone with Idiopathic Pulmonary Fibrosis?

A

•CXR : Basal/Posterior, Diffuse infiltrates, Cysts, ‘ Ground Glass ’

39
Q

What is UIP Basal and posterior fibrosis with honeycombing often complicated with?

A

Peripheral adenocarcinoma

40
Q

Describe the airway flow in interstitial lung disease

A

Normal air flow, diffusion is altered

41
Q

What is normal PaO2 and PaCO2?

A
42
Q

What is type 1 and type 2 respiration failure?

A
43
Q

What are the 4 abnormal states associated with Abnormal pulmonary gas exchange?

A

Alveolar hypoventilation

Shunt

Ventilation / Perfusion Imbalance

Diffusion Impairment

44
Q

What is the resut of alveolar hypoventilation on PACO2 and PAO2 ?

A

Hypoventilation increases the PACO2 and therefore decreases PaCO2

Increased PACO2 takes up space in the alveoli and therefore causes PAO2 and PaO2 to fall

45
Q

How can you correct a fall in PaO2 due to hypoventilation?

A

Raising the FIO2

46
Q

What is the commones cause of hypoxaemia clinically?

A

V/Q mismatch

47
Q

Why is normal V/Q ration 0.8?

A

•Normally breath 4 l/min. CO is 5 l/min so normal V/Q is 4/5 or 0.8

48
Q

How can you treat hypoxaemia due to low V/Q?

A

Increase in FIO2

49
Q

Will Interstitial lung disease affect CO2 levels?

A

Not usally becuase CO2 diffuses 20 times faster than O2

50
Q

How does diffusion impairment affect diffusing speed of oxygen?

A

•Diffusion impairment means it takes LONGER for blood and alveolar air to equilibrate, particularly for oxygen

51
Q

What is equilibration time for patients with diffusino impairment?

A

May take as long as 0.75 seconds - the same time as capillary transit time. Normally the equillibration takes 0.25 seconds.

52
Q

Why does diffusion impairment result in a fall in Pa O2 during exercise?

A

Capillary transit time decreases during exercise, less time for equillibration.

53
Q

Why don’t we have 100% haemoglobin saturation?

A

Because we have a 2-4% shunt

54
Q

When do we see a pathological shunt?

A

Alveolar ventilatino malformations, congenital heart disease and pulmonary disease

55
Q

Does large shunt respond well to increase in FIO2?

A

No, blood leaving normal sections of the lung is already 98% saturated

1st Year Systems (81 decks)

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