Restrictive Lung Disease

Question 1

What is the physiological definition of restrictive lung disease?

Answer 1

FVC is <80% of normal

Question 2

What volumes are affected in restrictive lung disease?

Answer 2

All are reduced - VC, RV, ERV, IC, TLC and TV

Question 3

What is the marker of restrictive lung disease?

Answer 3

Vital capacity
VC < 80% is abnormal

Question 4

What is used to determine FVC?

Answer 4

Spirometry

Question 5

What are some interstitial lung diseases?

Answer 5

Idiopathic pulmonary fibrosis
Sarcoidosis
Hypersensitivity pneumonitis

Question 6

What are some pleural causes of restrictive lung disease?

Answer 6

Pleural effusions
Pneumothorax
Pleural thickening

Question 7

Explain pleural effusions

Answer 7

There is an excessive collection of fluid in the pleural cavity
Has restricted pattern of lung airflow

Question 8

Explain a pneumothorax

Answer 8

A collapsed lung that occurs when air enters into the pleural cavity
This causes chest pain and breathlessness

Question 9

Explain pleural thickening

Answer 9

Increase of bulkiness of one or both of the pulmonary pleura
Can be due to asbestos exposure
TB can also cause pericardium thickening

Question 10

What are some skeletal causes for restrictive lung disease?

Answer 10

Kyphoscoliosis
Ankylosing spondylitis
Thoracoplasty
Rib fractures

Question 11

What is kyphoscoliosis?

Answer 11

An abnormality of the spine causing excessive curvature of the upper back. This causes pain and stiffness

Question 12

What is ankylosing spondylitis?

Answer 12

A type of arthritis in the spine, causing inflammation and gradual fusing of the vertebrae

Question 13

What is a thoracoplasty?

Answer 13

Thoracoplasty is a surgical technique initially designed to permanently collapse tuberculous cavities by resection of ribs from the chest wall
Volume in right is one side is increased and calcification can occur

Question 14

Explain rib fractures and restrictive lung condition?

Answer 14

If sore then cant expand rib
If in CF then can be dangerous as can bring up a lot of sputum

Question 15

Describe amyotrophic lateral sclerosis and restrictive lung disease?

Answer 15

This is caused by muscles
Muscles cant inflate lungs

Question 16

What are some sub-diaphragmic causes of restrictive lung disease?

Answer 16

Obesity and pregnancy

Question 17

Where is the interstitium?

Answer 17

It is between the epithelium of the alveolus and endothelium of the capillary

Question 18

Describe the interstitium?

Answer 18

Is very thin and if thickened can cause shortness of breath

Question 19

What are interstitial lung diseases?

Answer 19

There are over 200 diseases causing thickening of the interstitium and can result in pulmonary fibrosis

Question 20

What is sarcoidosis?

Answer 20

Multisystem granulomatous disease of unknown cause
Granuloma is a cluster of inflammatory cells

Question 21

What is the histological hallmark of sarcoidosis?

Answer 21

Non-caseating granuloma

Question 22

What is Erythema nodosum?

Answer 22

Is reddish, painful, tender lumps most commonly located in the front of the legs
Is skin inflammation
Patients with sarcoidosis can present with this rash

Question 23

What is anterior uveitis?

Answer 23

Inflammation of the iris and ciliary body - not middle of eye
Painful red eye

Question 24

Describe skin lesions and sarcoidosis?

Answer 24

Skin lesions are granulomatous so show hallmark of sarcoidosis

Question 25

Where do granulomas tend to be located?

Answer 25

At areas of skin trauma - scars or tattoos

Question 26

What is lupus pernio?

Answer 26

Is a nasal lesion which can be a clinical presentation of chronic sarcoidosis Treated with steroid injections and other therapies

Question 27

Who usually gets sarcoidosis?

Answer 27

Adults under 40 years old More women then men Happens world wide

Question 28

How do we investigate sarcoidosis?

Answer 28

History and exam CXR Then pulmonary function tests, bloods, urinalysis, ECG, TB skin test and eye exam

Question 29

What would further investigation of sarcoidosis involve?

Answer 29

Bronchoscopy including transbronchial biopsies and endobronchial US

Question 30

What surgical biopsies may be needed in sarcoidosis?

Answer 30

Mediastinoscopy Video assisted thoracoscopy lung biopsy (VATS) - uncommon

Question 31

Describe stage 1 -4 of sarcoidosis on CXR?

Answer 31

1 - bulky lymph glands - paratracheal nodes 2 - spotty lungs 3 - interstitial change 4 - fibrotic advancement

Question 32

Does stage affect prognosis?

Answer 32

Stage 1 has highest of 55-90% Then lower is stage 4 is 0% as now fibrotic change which is irreversible

Question 33

How should sarcoidosis be treated in patient with mild disease, no vital organ involvement and normal lung function?

Answer 33

No treatment

Question 34

How should you treat a patient with erythema nodosum/ arthralgia?

Answer 34

NSAIDs - non-steroidal anti-inflammatory drugs

Question 35

How would you treat a patient with skin lesions/ anterior uveitis or cough?

Answer 35

Tropical steroids rather than systemic drugs

Question 36

What is the treatment for a patient who has cardiac, neurological sarcoid, eye disease not responding to topical Rx?

Answer 36

Systemic steroids ex. prednisolone Also used if patient has hypercalcaemia

Question 37

What are some respiratory complications of sarcoidosis?

Answer 37

Progressive resp. failure Bronchiectasis Aspergilloma can give haemoptysis or pneumothorax

Question 38

What is idiopathic pulmonary fibrosis?

Answer 38

Is chronic scarring lung disease and breathing becomes increasingly more difficult Gives the honeycomb lung Has fibroblastic focus - collagen factory

Question 39

What is the typical presentations of IPF?

Answer 39

Chronic breathlessness, cough, clubbed and crackles Failed prescription for LVF or infection Typically 60-70 can commoner in men

Question 40

What are the options of management for a patient with IPF?

Answer 40

Refer to ILD clinic Medical options - oral anti-fibrotic (prifenidone, nintedanib) and palliative care Transplant

Question 41

What is hypersensitivity pneumonitis?

Answer 41

When lungs develop an immune response to something you breath in and causes inflammation of lung tissue

Question 42

What are the causes of hypersensitivity pneumonitis?

Answer 42

Birds and pigeons Hay Malted whiskey factory - lungs reacting against factory