Clinical Features and Management of Restrictive Lung Diseases

Question 1

What is the physiological definition of restriction?

Answer 1

Forced vital capacity <80% of normal

Question 2

What is a marker of restriction?

Answer 2

Vital capacity

Question 3

What is used to determine restriction rates?

Answer 3

Spirometry

Question 4

What vital capacity value is considered to be abnormal?

Answer 4

<80%

Question 5

Where might there be restriction?

Answer 5

Lungs
Pleura
Muscle/nerve
Bone
Other

(think staring in the middle and work your way out).

Question 6

List some interstitial lung diseases.

Answer 6

Idiopathic pulmonary fibrosis
Sarcoidosis
Hypersensitivity pneumonitis

Question 7

List some of the pleural causes of restriction

Answer 7

Pleural effusion
Pleural thickening
Pneumothorax

Question 8

List some of the skeletal causes of restriction.

Answer 8

Kyphoscoliosis
Ankylosing spondylitis
Thoracoplasty
Rib fractures

Question 9

What is Thoracoplasty?

Answer 9

Surgery removing some rib segments.

->don’t necessarily need to know, just to aid understanding

Question 10

What is Ankylosing spondylitis ?

Answer 10

Inflammation of the spine.

->don’t necessarily need to know, just to aid understanding

Question 11

What is Kyphoscoliosis?

Answer 11

Abnormal curvature of the spine.

->don’t necessarily need to know, just to aid understanding

Question 12

Name a muscle causes of restriction.

Answer 12

Amyotrophic lateral sclerosis (ms)

Question 13

Name some sub-diaphragmatic causes of restriction.

Answer 13

Obesity
Pregnancy

Question 14

What is the interstitium?

Answer 14

Tiny space between the epithelium of the alveolus and the endothelium of the capillary

Question 15

What can happen to a patient if there is an abnormality in the interstitium?

Answer 15

Can cause breathlessness

Question 16

What happens to the Interstitium to cause breathlessness in a patient?

Answer 16

It becomes thickened/enlarged.

Question 17

What are interstitial lung diseases?

Answer 17

Diseases causing thickening of the interstitium and can result in pulmonary fibrosis

Question 18

What is sarcoidosis?

Answer 18

Interstitial lung disease
Multisystem granulomatous disease of unknown cause

Question 19

What is the histological landmark in sarcoidosis?

Answer 19

Non-caseating granulomas (without necrosis)

Question 20

What is a classical presentation for someone with sarcoidosis?

Answer 20

Red, raised, painful legion on the legs

Question 21

Where will granulomas often locate?

Answer 21

Areas of skin trauma e.g. scars, tattoos

Question 22

Who tends to get sarcoidosis?

Answer 22

Adults <40
Women get it more than men

Question 23

What are the investigations for sarcoidosis?

Answer 23

History and examination
Chest x-ray

Question 24

Which surgical biopsies may be required for somebody with sarcoidosis?

Answer 24

Mediastinoscopy
Video assisted thoracoscopic lung biopsy (VATS)

Question 25

What is the treatment for someone with sarcoidosis when they present as following- Mild disease, no vital organ involvement, normal lung function, few symptoms

Answer 25

No treatment

Question 26

What is the treatment for someone with sarcoidosis when they present as following- Erythema nodosum / arthralgia

Answer 26

NSAIDs

Question 27

What is the treatment for someone with sarcoidosis when they present as following- Skin lesions / anterior uveitis / cough

Answer 27

Topical steroids

Question 28

What is the treatment for someone with sarcoidosis when they present as following- Cardiac, neurological, eye disease not responding to topical Rx, hypercalcaemia

Answer 28

Systemic steroids

Question 29

What are some of the respiratory complications which may arise in someone with sarcoidosis?

Answer 29

Progressive respiratory failure Bronchiectasis Aspergilloma, haemoptysis, pneumothorax

Question 30

What is the typical presentation for someone with Idiopathic pulmonary fibrosis (IPF)?

Answer 30

Chronic breathlessness & cough Clubbed & crackles

Question 31

Describe the people which are more likely to develop IPF.

Answer 31

60-70 years old More common in men

Question 32

What is the average survival rate of IPF?

Answer 32

3 years

Question 33

Name some treatment options for those w IPF which although cannot cure, can slow the disease.

Answer 33

Drugs Palliative care Surgical transplant

Question 34

What is hypersensitive pneumonitis?

Answer 34

Developing an immune response to something breathed in and causing inflammation of the airways.

Question 35

What can hypersensitive pneumonitis be caused by?

Answer 35

Birds- by breathing in particles from feathers of birds/bird droppings Gran/stray/hay- breathing in a mould which may grow on it