Restrictive lung disease - clinical features and management Flashcards

1
Q

What is the physiological definition of restriction?

A

A forced vital capacity <80% of the predicted value

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2
Q

Working from inside to outside what should you think of when it comes to restriction?

A

lungs-> pleura->nerve or muscle-> bone and other

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3
Q

Name the Main lung causes of restriction

A

interstitial lung diseases

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4
Q

Name the 3 main interstitial lung diseases

A

idiopathic pulmonary fibrosis
sarcoidosis
hypersensitivity pneumonitis

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5
Q

Name 3 pleural causes of restriction

A

pneumothorax
pleural effusion
pleural thickening

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6
Q

Name 4 bone/skeletal causes of restriction

A

Kyphoscoliosis
ankylosing spondylitis
thoracoplasty
rib fractures

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7
Q

Name a muscle cause of restriction

A

amyotrophic lateral sclerosis

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8
Q

Name some other sub diaphragmatic causes of restriction

A

obesity

pregnancy

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9
Q

Where is the interstitium?

A

Space between the epithelium of the alveolus and the endothelium of the capillary

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10
Q

What are interstitial lung diseases?

A

Diseases causing thickening of the interstitium

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11
Q

How many ILDs are there?

A

> 200

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12
Q

What can ILDs result in?

A

pulmonary fibrosis

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13
Q

What is pleural thickening related to?

A

asbestos exposure

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14
Q

What is sarcoidosis?

A

multisystem granulomatous disease of unknown cause

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15
Q

What is the histological hallmark of sarcoidosis?

A

non caseating granuloma

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16
Q

Who does sarcoidosis most commonly affect?

A

females under 40

17
Q

Name some signs of sarcoidosis

A

red painful ankles and skin lesions - erythema nodosum
anterior uveitis
Granulomas collecting at scar tissue

18
Q

List some investigations done for sarcoidosis

A

CXR, urine samples, eye test, TB test, bloods, ECG and pulmonary function

19
Q

What is the further assessment for sarcoidosis?

A

bronchoscopy - including biopsies and ultrasound

20
Q

What surgical biopsies may be needed for sarcoidosis?

A

mediastinoscopy

video assisted thoracoscopic lung biopsy

21
Q

For mild sarcoidosis what is the treatment?

A

none

22
Q

For sarcoidosis with erythema nodosum and arthralgia what is the treatment?

A

NSAIDs

23
Q

For sarcoidosis with skin lesions and uveitis what is the treatment?

A

topical steroids

24
Q

For sarcoidosis with cardiac and neurological involvement what is the treatment?

A

systemic steroids

25
Q

About what percentage of Caucasians die from sarcoidosis?

A

<1%

26
Q

What percentage of people sustain permanent (extra) pulmonary complications due to sarcoidosis?

A

10-20%

27
Q

Name some respiratory complications sustained after sarcoidosis

A

progressive respiratory failure
bronchiectasis
haemoptysis and pneumothorax

28
Q

What is the ususal circumstances for IPF?

A

Males 60-70
chronic dyspnoea and cough
clubbing

29
Q

What is the median survival for IPF?

A

3 years

30
Q

What are the medical options for IPF?

A

Oral anti fibrotics

31
Q

What are the surgical options for IPF?

A

transplant