Clinical Features and Management of Restrictive Lung Disease Flashcards

1
Q

What is the physiological definition of restriction?

A

Forced vital capacity less than 80% of the predicted normal

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2
Q

What is a marker of restriction?

A

Vital capacity

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3
Q

What are causes of restriction?

A

Lungs

Pleura

Nerve

Muscle

Bone

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4
Q

What are lung causes of restriction?

A

Interstitial lung diseases

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5
Q

What are examples of interstitial lung diseases?

A

Idiopathic pulmonary fibrosis

Sarcoidosis

Hypersensitivity pneumonitis

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6
Q

What are pleural causes of restriction?

A

Pleural effusions

Pneomothorax

Pleural thickening

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7
Q

What are skeletal causes of restriction?

A

Kyphoscoliosis

Ankylosing spondylitis

Thoracoplasty

Rib fractures

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8
Q

What are muscle causes of restriction?

A

Amyotrophic lateral sclerosis

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9
Q

What are sub-diaphragmatic causes of restriction?

A

Obesity

Pregnancy

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10
Q

What should you remember about the causes of restriction?

A

Not always lung disease

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11
Q

Where is the lung interstitium?

A

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

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12
Q

What does ILD stand up for?

A

Interstitium lung disease

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13
Q

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

A

More than 200

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14
Q

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

A

Sarcoidosis

Idiopathic pulmonary fibrosis

Hypersensitive pneumonitis

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15
Q

What is sarcoidosis?

A

Multisystem granulomatous disease of unknown cause

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16
Q

What age and sex commonly gets sarcoidosis?

A

Younger than 40

Women more than men

17
Q

How do you investigate sarcoidosis?

A

History and examination

Chest X-ray

Pulmonary function tests

Bloods

18
Q

What further assessments can be done to investigate sarcoidosis?

A

Bronchoscopy including transbronchial biopsies and endobronchial ultrasound

19
Q

What surgical biopsies can be done for sarcoidosis?

A

Mediastinoscopy

Video assisted thoracoscopic lung biopsy

20
Q

What does VATS stand up for?

A

Video assisted thoracoscopic lung biopsy

21
Q

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

A

No treatment

22
Q

How should sarcoidosis be treated if erythema nodosum/arthralgia?

A

NSAIDS

23
Q

What is erythema nodosum?

A

Swollen fat under the skin causing red bumps

24
Q

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

A

Erythema nodosum

25
Q

What is arthralgia?

A

Pain in a joint

26
Q

What is pain in a joint called?

A

Arthralgia

27
Q

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

A

Topical steroids

28
Q

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

A

Systemic steroids

29
Q

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

A

Progressive respiratory failure

Bronchiectasis

Aspergilloma

Pneumothorax

30
Q

What percentage of caucasians die from sarcoidosis?

A

<1%

31
Q

What is the typical presentation of idiopathic pulmonary fibrosis?

A

Chronic breathlessness and cough

Typically 60-70 years old

Clubbed and crackles

32
Q

What is idiopathic pulmonary fibrosis?

A

Scarring of the lung

33
Q

What is scarring of the lung for unknown causes called?

A

Idiopathic pulmonary fibrosis

34
Q

What is the median survival time for idiopathic pulmonary fibrosis?

A

3 years

35
Q

What are the options for pulmonary fibrosis?

A

Refer to interstitial lung disease clinic

Palliative care

Transplant

36
Q

What is hypersensitive pneumonitis?

A

Lungs become inflammed due to an allergic reaction

37
Q

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

A

Hypersensitive pneumonitis