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Respiratory block > Clinical Features and Management of Restrictive Lung diseases > Flashcards

Clinical Features and Management of Restrictive Lung diseases Flashcards

1
Q

What is the physiological definition of restriction ?

A

FVC <80% of normal

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2
Q

What is the marker of restriction ?

A

Vital capacity- marker of restriction

Spirometry is key

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3
Q

What are the lung causes of Interstitial lung diseases ?

A

Interstitial lung disease:

  - idiopathic pulmonary fibrosis 
  - sarcoidosis 
  - hypersensitivity pneumonitis
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4
Q

What are the pleural causes of restrictive lung disease ?

A

Pleural effusions
Pneumothorax
Pleural thickening

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5
Q

What are the skeletal causes of restrictive lung diseases ?

A

Kyphoscoliosis
Ankylosing spondylitis
Thoracoplasty
Rib fractures - soreness

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6
Q

What are the muscular and nerve causes of restrictive lung disease ?

A

Amyotrophic lateral sclerosis

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7
Q

What are the sub-diaphragmatic causes of restrictive lung disease ?

A

Obesity

Pregnancy

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8
Q

What is sarcoidosis ?

A

Multisystem granulomatous disease of unknown cause

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9
Q

What is the histological hallmark of sarcoidosis ?

A

Non-caseating granuloma

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10
Q

What are the clinical signs of sarcoidosis ?

A

Erythema nodosum - granulomas often locate to scar tissue

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11
Q

Who gets sarcoidosis ?

A

Adults <40yr
F>M
Worldwide

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12
Q

How do you investigate sarcoidosis ?

A

History and exam
CXR
Bronchoscopy including transbronchial biopsies and endobronchial ultrasound
Pulmonary function tests
Bloods / urinalysis / ECG / TB skin test / eye exam
Surgical biopsies:
- mediastinoscopy
- Video assisted thoracoscopic lung biopsy

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13
Q

What is the remission rates for stage 1-4 sarcoidosis ?

A

Stage 1 - 55-90%
Stage 2 - 40-70%
Stage 3 - 10-20%
Stage 4 - 0%

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14
Q

When would you provide no treatment for Sarcoidosis ?

A

Mild disease, no vital organ involvement, normal lung function, few symptoms

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15
Q

When would you treat sarcoidosis with NSAIDs?

A

Erythema nodosum / arthralgia

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16
Q

When would you treat sarcoidosis with Topical steroids ?

A

Skin lesions / anterior uveitis / cough

17
Q

When would you treat sarcoidosis with Systemic Steroids ?

A

Cardiac, neurological, eye disease not responding to topical Rx, hypercalcaemia

18
Q

What is prognosis ?

A

10-20% sustain permenant pulmonary or extrapulmonary complications

19
Q

What is the typical presentation for idiopathic pulmonary fibrosis ?

A 
Chronic breathlessness & cough
Typically 60-70 yrs old
M>F
Failed Rx for LVF or infection
Clubbed & crackles
20
Q

What are the options for Idiopathic pulmonary fibrosis ?

A

Median survival - 3 years
Refer to ILD clinic
Medical options- OAF (oral anti-fibrotic)- Pirfenidone
Surgical option- treatment

21
Q

What initiates hypersensitivity pneumonitis ?

A

Allergens from things like, pigeons, mould, farms etc

Respiratory block (38 decks)

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