Restrictive Lung Disease Flashcards

1
Q

What is the physiological definition of restrictive lung disease?

A

FVC is <80% of normal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What volumes are affected in restrictive lung disease?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is the marker of restrictive lung disease?

A

Vital capacity
VC < 80% is abnormal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is used to determine FVC?

A

Spirometry

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are some interstitial lung diseases?

A

Idiopathic pulmonary fibrosis
Sarcoidosis
Hypersensitivity pneumonitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are some pleural causes of restrictive lung disease?

A

Pleural effusions
Pneumothorax
Pleural thickening

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Explain pleural effusions

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Explain a pneumothorax

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Explain pleural thickening

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are some skeletal causes for restrictive lung disease?

A

Kyphoscoliosis
Ankylosing spondylitis
Thoracoplasty
Rib fractures

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is kyphoscoliosis?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is ankylosing spondylitis?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is a thoracoplasty?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Explain rib fractures and restrictive lung condition?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Describe amyotrophic lateral sclerosis and restrictive lung disease?

A

This is caused by muscles
Muscles cant inflate lungs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

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

A

Obesity and pregnancy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Where is the interstitium?

A

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

18
Q

Describe the interstitium?

A

Is very thin and if thickened can cause shortness of breath

19
Q

What are interstitial lung diseases?

A

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

20
Q

What is sarcoidosis?

A

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

21
Q

What is the histological hallmark of sarcoidosis?

A

Non-caseating granuloma

22
Q

What is Erythema nodosum?

A

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

23
Q

What is anterior uveitis?

A

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

24
Q

Describe skin lesions and sarcoidosis?

A

Skin lesions are granulomatous so show hallmark of sarcoidosis

25
Q

Where do granulomas tend to be located?

A

At areas of skin trauma - scars or tattoos

26
Q

What is lupus pernio?

A

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

27
Q

Who usually gets sarcoidosis?

A

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

28
Q

How do we investigate sarcoidosis?

A

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

29
Q

What would further investigation of sarcoidosis involve?

A

Bronchoscopy including transbronchial biopsies and endobronchial US

30
Q

What surgical biopsies may be needed in sarcoidosis?

A

Mediastinoscopy
Video assisted thoracoscopy lung biopsy (VATS) - uncommon

31
Q

Describe stage 1 -4 of sarcoidosis on CXR?

A

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

32
Q

Does stage affect prognosis?

A

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

33
Q

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

A

No treatment

34
Q

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

A

NSAIDs - non-steroidal anti-inflammatory drugs

35
Q

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

A

Tropical steroids rather than systemic drugs

36
Q

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

A

Systemic steroids ex. prednisolone
Also used if patient has hypercalcaemia

37
Q

What are some respiratory complications of sarcoidosis?

A

Progressive resp. failure
Bronchiectasis
Aspergilloma can give haemoptysis or pneumothorax

38
Q

What is idiopathic pulmonary fibrosis?

A

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

39
Q

What is the typical presentations of IPF?

A

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

40
Q

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

A

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

41
Q

What is hypersensitivity pneumonitis?

A

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

42
Q

What are the causes of hypersensitivity pneumonitis?

A

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