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Flashcards in Pathology of Restrictive Lung Disease Deck (105)
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1

What is the interstitium of the lung?

Connective tissue space around the airways and vessels and the space between the basement membranes of the alveolar walls

2

What is the connective tissue space around the airways and vessels called?

Interstitium of the lung

3

How are alveolar epithelial (pneumocytes) and interstitial capillary endothelial cell basement membranes in normal alveolar walls?

In direct contact

4

What does restrictive lung disease show?

Reduced lung compliance (stiff lungs)

Low FEV1 and low FVC but same FEV1/FVC ratio

Reduced gas transfer

Ventilation/perfusion mismatch

5

What does the spirometry of restrictive lung disease look like?

6

What is the presentation of restrictive lung disease?

Abnormal chest X-ray

Dyspnoea (on exertion and rest))

Respiratory failure (type 1)

Heart failure

7

What is dyspnoea?

Difficult breathing

8

What is difficult breathing called?

Dyspnoea

9

What is the difference in chest X-rays between:

Emphysema
Normal
Restrictive lung disease

Emphysema can see all ribs

Normal can see 10 ribs

Restrictive lung disease can see less than 10

10

What is restrictive lung disease also known as?

Interstitial lung disease

11

What is the pathway leading to restrictive lung disease?

1) Lung injury

2) Leads to chronic response

3) One of usual interstitial pneumonitis (UIP), granulomatous response, or other pattens leads to

4) Fibrosis or end stage honeycomb lung

12

What does the acute response to lung injury lead to?

Diffuse alveolar damage

13

What is diffuse alveolar damage associated with?

Major trauma

Chemical injury/toxic inhalation

Circulatory shock

Drugs

Infection

Autoimmune disease

Radiation

Idiopathic

14

What does idiopathic mean?

No known cause

15

What is no known cause called?

Idiopathic

16

What does the extend of diffuse alveolar damage depend on?

How many days the injury lasts for

17

What does DADS stand up for?

Diffuse alveolar damage

18

What are the histological features of acute alveolar damage?

Protein rich oedema

Fibrin

Hyaline membranes

Denuded basement membranes

Epithelial proliferation

Fibroblast proliferation

Scarring

19

Why do granulomatous responses to chronic inflammation lead to fibrosis or end stage honeycomb lung?

Sarcoidosis

Hypersensitivity pneumonitis

20

What is sarcoidosis?

Multisystem granulomatous disorder of unknown aetiology

21

What is a multisystem granulomatous disorder of known aetiology?

Sarcoidosis

22

What is the histopathology of sarcoidosis?

Epitheloid and giant cell granulomas

Necrosis/caseation very unusual

Little lymphoid infiltrate

Variable associated fibrosis

23

What is a granulomas?

Structure formed during inflammation that is found in many diseases, being a collection of macrophages

24

What is a structure formed during inflammation that is composed of a collection of macrophages?

Granulomas

25

What is the purpose of a granulomas?

Wall of a substance that cannot be destroyed

26

What is used to wall of a substance that cannot be destroyed?

Granulomas

27

Who does sarcoidosis commonly affect?

Young adults

28

Does sarcoidosis affect more males or females?

Females

29

What is the prevalence of sarcoidosis?

3-4/100,000 in UK

30

Where is sarcoidosis often common?

Temperate climates