Clinical features and Management of Restrictive Lung Diseases COPY Flashcards

1
Q

What is the physiological definition of restriction?

A

Forced vital capacity of less than 80% of predicted normal

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2
Q

Describe the differences between the pulmonary function tests between normal, obstructive and restrictive lung

A

Obstructive

  • Larger TLC, RV, FRC
  • Smaller VC, TV, IC, ERV

Restrictive

  • Smaller TLC, VC, IC, TV, ERV, FRC, RV
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3
Q

What is the marker of restrictinon?

A

Vital capacity - spirometry

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4
Q

What are the lung causes of Interstitial lung diseases?

A
  • Idiopathic pulmonary fibrosis
  • Sarcoidosis
  • Hypersensitivity pneumonitis
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5
Q

What are the pleural causes of restrictive lung disease?

A

Pleural effusion

Pneumothorax

Pleural thickening - asbestos related, pneumonia

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6
Q

What are the skeletal causes of restrictive lung diseases?

A

Kyphoscoliosis

Ankylosing spondylitis

Thoracoplasty

Rib fractures - soreness

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7
Q

What are the muscular casues of restrictive lung disease?

A

Amyotrophic lateral sclerosis

Obesity - sub diaphragmatic - diaphragm can’t fall properly

Pregnancy - sub diaphragmatic

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8
Q

What is the interstitium?

A

Space between alveoli and capillary

Between alveolar epithelium and capillary endothelium

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9
Q

What is sarcoidosis?

A

Multisystem granulomatous disease of unknown cause

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10
Q

What is the Histological hallmark of Sarcoidosis?

A

non-caseating granuloma

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11
Q

What are the clinical signs of sarcoidosis?

A

Erythema nodosum - granulomas often locate to scar tissue

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12
Q

Who gets sarcoidosis?

A

Adults < 40

Women more than men

World-wide

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13
Q

How do you investigate sarcoidosis?

A

History and exam

CXR

Pulmonary function tests

Bloods / urinalysis / ECG / TB skin test / eye exam

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14
Q

What would the further assessment of sarcoidosis be?

A

Bronchoscopy including transbronchial biopsies and endobronchial ultrasound

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15
Q

What would be the different surgical biopsy options for sarcoidosis

A

Mediastinoscopy

Video assisted thoracoscopic lung biopsy (VATS)

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16
Q

Look at the different stages as presented on these chest X-Rays

A
17
Q

When would you provide no treatment for Sarcoidosis?

A
  • Mild disease
  • no vital organ involvement
  • normal lung function
  • few symptoms
18
Q

When would you treat sarcoidosis with NSAIDS?

A

Erythema nodosum / arthralgia

19
Q

When would you treat sarcoidosis with Topical steroids?

A

Skin lesions / anterior uveitis / cough

20
Q

When would you treat sarcoidosis with Systemic Steroids?

A

If Cardiac, neurological, eye disease not responding to topical steroids, or in hypercalcaemia

21
Q

What is prognosis?

A

Few Caucasian’s die (<1%)

10-20% get permanent pulmonary/extra-pulmonary complications

Respiratory:

  • Progressive respiratory failure
  • Bronchiectasis
  • Aspergilloma, haemoptysis, pneumothorax
22
Q

What is the typical presentation for idiopathic pulmonary fibrosis?

A
  • Chronic breathlessness & cough
  • Typically 60-70 years old, commoner in men
  • Predisposed to lung cancer
  • Clubbed & crackles
23
Q

How does fibrotic tissue vary in the lung?

A

Usually most prominent in peripheral tissue, central tissue is usually unaffected

Worsens as severity of condition increases

24
Q

What are the options for Idiopathic pulmonary fibrosis?

A

Median survival 3 y.

  • refer to ILD clinic

Medical

  • OAF (oral anti fibrotic) – slow down progression but does NOT stop it
  • palliative care

Surgical

Transplant

25
Q

What initiates hypersensitivity pneumonitis?

A

Allergens from, pigeons, mould, farms etc