Clinical Features and Management of Restrictive Lung Diseases Flashcards

1
Q

What is the physiological definition of restriction?

A

Forced vital capacity <80% of normal

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2
Q

What is a marker of restriction?

A

Vital capacity

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3
Q

What is used to determine restriction rates?

A

Spirometry

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4
Q

What vital capacity value is considered to be abnormal?

A

<80%

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5
Q

Where might there be restriction?

A

Lungs
Pleura
Muscle/nerve
Bone
Other

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

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6
Q

List some interstitial lung diseases.

A

Idiopathic pulmonary fibrosis
Sarcoidosis
Hypersensitivity pneumonitis

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7
Q

List some of the pleural causes of restriction

A

Pleural effusion
Pleural thickening
Pneumothorax

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8
Q

List some of the skeletal causes of restriction.

A

Kyphoscoliosis
Ankylosing spondylitis
Thoracoplasty
Rib fractures

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9
Q

What is Thoracoplasty?

A

Surgery removing some rib segments.

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

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10
Q

What is Ankylosing spondylitis ?

A

Inflammation of the spine.

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

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11
Q

What is Kyphoscoliosis?

A

Abnormal curvature of the spine.

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

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12
Q

Name a muscle causes of restriction.

A

Amyotrophic lateral sclerosis (ms)

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13
Q

Name some sub-diaphragmatic causes of restriction.

A

Obesity
Pregnancy

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14
Q

What is the interstitium?

A

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

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15
Q

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

A

Can cause breathlessness

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16
Q

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

A

It becomes thickened/enlarged.

17
Q

What are interstitial lung diseases?

A

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

18
Q

What is sarcoidosis?

A

Interstitial lung disease
Multisystem granulomatous disease of unknown cause

19
Q

What is the histological landmark in sarcoidosis?

A

Non-caseating granulomas (without necrosis)

20
Q

What is a classical presentation for someone with sarcoidosis?

A

Red, raised, painful legion on the legs

21
Q

Where will granulomas often locate?

A

Areas of skin trauma e.g. scars, tattoos

22
Q

Who tends to get sarcoidosis?

A

Adults <40
Women get it more than men

23
Q

What are the investigations for sarcoidosis?

A

History and examination
Chest x-ray

24
Q

Which surgical biopsies may be required for somebody with sarcoidosis?

A

Mediastinoscopy
Video assisted thoracoscopic lung biopsy (VATS)

25
Q

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

A

No treatment

26
Q

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

A

NSAIDs

27
Q

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

A

Topical steroids

28
Q

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

A

Systemic steroids

29
Q

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

A

Progressive respiratory failure
Bronchiectasis
Aspergilloma, haemoptysis, pneumothorax

30
Q

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

A

Chronic breathlessness & cough
Clubbed & crackles

31
Q

Describe the people which are more likely to develop IPF.

A

60-70 years old
More common in men

32
Q

What is the average survival rate of IPF?

A

3 years

33
Q

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

A

Drugs
Palliative care
Surgical transplant

34
Q

What is hypersensitive pneumonitis?

A

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

35
Q

What can hypersensitive pneumonitis be caused by?

A

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