Clinical Features and Management of Restrictive Lung Disease

Question 1

What is the physiological definition of restriction?

Answer 1

Forced vital capacity less than 80% of the predicted normal

Question 2

What is a marker of restriction?

Answer 2

Vital capacity

Question 3

What are causes of restriction?

Answer 3

Lungs

Pleura

Nerve

Muscle

Bone

Question 4

What are lung causes of restriction?

Answer 4

Interstitial lung diseases

Question 5

What are examples of interstitial lung diseases?

Answer 5

Idiopathic pulmonary fibrosis

Sarcoidosis

Hypersensitivity pneumonitis

Question 6

What are pleural causes of restriction?

Answer 6

Pleural effusions

Pneomothorax

Pleural thickening

Question 7

What are skeletal causes of restriction?

Answer 7

Kyphoscoliosis

Ankylosing spondylitis

Thoracoplasty

Rib fractures

Question 8

What are muscle causes of restriction?

Answer 8

Amyotrophic lateral sclerosis

Question 9

What are sub-diaphragmatic causes of restriction?

Answer 9

Obesity

Pregnancy

Question 10

What should you remember about the causes of restriction?

Answer 10

Not always lung disease

Question 11

Where is the lung interstitium?

Answer 11

Between the epithelium of the alveoli and the endothelium of the capillary

Question 12

What does ILD stand up for?

Answer 12

Interstitium lung disease

Question 13

How many diseases cause the thickening of the interstitium and can result in pulmonary fibrosis?

Answer 13

More than 200

Question 14

What are the top 3 lung diseases that cause interstitium lung disease?

Answer 14

Sarcoidosis

Idiopathic pulmonary fibrosis

Hypersensitive pneumonitis

Question 15

What is sarcoidosis?

Answer 15

Multisystem granulomatous disease of unknown cause

Question 16

What age and sex commonly gets sarcoidosis?

Answer 16

Younger than 40

Women more than men

Question 17

How do you investigate sarcoidosis?

Answer 17

History and examination

Chest X-ray

Pulmonary function tests

Bloods

Question 18

What further assessments can be done to investigate sarcoidosis?

Answer 18

Bronchoscopy including transbronchial biopsies and endobronchial ultrasound

Question 19

What surgical biopsies can be done for sarcoidosis?

Answer 19

Mediastinoscopy

Video assisted thoracoscopic lung biopsy

Question 20

What does VATS stand up for?

Answer 20

Video assisted thoracoscopic lung biopsy

Question 21

How should sarcoidosis be treated if mild disease with no vital organ involvement, normal lung function and few symptoms?

Answer 21

No treatment

Question 22

How should sarcoidosis be treated if erythema nodosum/arthralgia?

Answer 22

NSAIDS

Question 23

What is erythema nodosum?

Answer 23

Swollen fat under the skin causing red bumps

Question 24

What is swollen fat under the skin causing red bumps called?

Answer 24

Erythema nodosum

Question 25

What is arthralgia?

Answer 25

Pain in a joint

Question 26

What is pain in a joint called?

Answer 26

Arthralgia

Question 27

How should sarcoidosis be treated if skin lesions and a cough are present?

Answer 27

Topical steroids

Question 28

How should sarcoidosis be treated if cardiac, neurological and eye disease not responding to topical steroids, or are hypercalcaemia?

Answer 28

Systemic steroids

Question 29

What are some permanent pulmonary complications that can occur due to sarcoidosis?

Answer 29

Progressive respiratory failure Bronchiectasis Aspergilloma Pneumothorax

Question 30

What percentage of caucasians die from sarcoidosis?

Answer 30

\<1%

Question 31

What is the typical presentation of idiopathic pulmonary fibrosis?

Answer 31

Chronic breathlessness and cough Typically 60-70 years old Clubbed and crackles

Question 32

What is idiopathic pulmonary fibrosis?

Answer 32

Scarring of the lung

Question 33

What is scarring of the lung for unknown causes called?

Answer 33

Idiopathic pulmonary fibrosis

Question 34

What is the median survival time for idiopathic pulmonary fibrosis?

Answer 34

3 years

Question 35

What are the options for pulmonary fibrosis?

Answer 35

Refer to interstitial lung disease clinic Palliative care Transplant

Question 36

What is hypersensitive pneumonitis?

Answer 36

Lungs become inflammed due to an allergic reaction

Question 37

What is it called when the lungs become inflammed due to an allergic reaction?

Answer 37

Hypersensitive pneumonitis