Pulmonary Pathophysiology - Restrictive Lung Disease Flashcards

1
Q

Restrictive lung disease definition

A

Diseases in which the expansion of the lung is restricted either because of alterations in the lung parenchyma, diseases of the pleura, chest wall or neuromusculature

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2
Q

Lung parenchyma definition

A

Gas exchanging lung tissue

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3
Q

Blood gas barrier diagram

A
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4
Q

Structure of type I collagen in alveolar wall

A

Forms type 1 collagen cable to support structure

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5
Q

Diffuse interstitial pulmonary fibrosis: alternate names

A

Idiopathic interstitial fibrosis
Interstitial pneumonia
Cryptogenic fibrosing alveolitis

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6
Q

Pathology of diffuse interstitial pulmonary fibrosis

A

Thickening of interstitium of alveolar wall

Initial infiltration with lymphocytes and plasma cells

Later fibroblasts lay down thick collagen bundles

Alveoli may contain cellular exudate ‘desquamation’

Eventually architecture is destroyed ith extensive scarring, dilated air spaces and ‘honey-combing’

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7
Q

Scarring definition

A

End result of many pathological processes
Due to laying down of collagen

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8
Q

Clinical features of Diffuse Interstitial Pulmonary Fibrosis

A

Not common

SOB with rapid shallow breathing

Reduced exercise tolerance

Dry cough

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9
Q

Signs of diffuse interstitial pulmonary fibrosis

A

Fine crepitations
Finger clubbing
Reduced chest movements

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10
Q

Radiological features of diffuse interstitial pulmonary fibrosis

A

CXR - small lungs with reticular pattern

Honey-comb appearance in late disease / on CT scan

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11
Q

Ventilatory mechanics and volume changes in restrictive lung disease

A

Low FEV1 and low FVC
Normal or increased FEV/FVC ratio

All lung volumes reduced

Reduced lung compliance

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12
Q

Changes to calibre of alveoli / small airways in pulmonary fibrosis

A

Calibre increases due to radial traction forces

Therefore have higher initial flow rates

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13
Q

Gas exchange changes in diffuse interstitial pulmonary fibrosis

A

Reduced PaO2
Normal or low PaCO2

PaO2 falls on exercise

Reduced diffusing capacity

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14
Q

Pulmonary circulation changes in diffuse interstitial pulmonary fibrosis

A

Many capillaries are obliterated by fibrosis

Pulmonary vascular resistance increases

Pulmonary hypertension - especially on exercise

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15
Q

Possible trigger for breathing with diffuse interstitial pulmonary fibrosis

A

?stimulation of J receptors causing rapid shallow breathing

Especially in context of normal or low PaCO2

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16
Q

Primary cause of arterial hypoxaemia in diffuse interstitial pulmonary fibrosis

A

V/Q mismatch

17
Q

Diseases which can cause interstitial pulmonary fibrosis

A

Sarcoidosis
Hypersensitivity pneumonitis
Collagen diseases
Drugs
Oxygen toxicity
Poisons

18
Q

Features of Sarcoidosis

A

Characteristic non caseating granulomas
Affects several organs
Probable immunological basis
Can cause interstitial pulmonary fibrosis

19
Q

Features of Hypersensitivity pneumonitis

A

Disease of lung parenchyma due to hypersensitivity to inhaled organic dusts

Exposure is heavy

Acute disease causes SOB, fever and cough

Longstanding disease can cause interstitial pulmonary fibrosis

20
Q

Examples of causes of Hypersensitivity pneumonitis

A

Farmer’s lung - spores in mouldy hay

Bird breeder’s lung

21
Q

Collagen disease example

A

Systemic scleroderma

22
Q

Drugs which can cause interstitial pulmonary fibrosis

A

Bleomycin
Busulfan

23
Q

Poison example which can cause interstitial pulmonary fibrosis

A

Paraquat - a weed killer

24
Q

Causes of restrictive lung disease outside of the lung

A

Pleural diseases

Chest wall diseases

Neuromuscular diseases

25
Q

Pleural diseases which can cause restrictive lung disease

A

Pneumothorax
Pleural effusion
Pleural thickening

26
Q

Chest wall diseases which can cause restrictive lung disease

A

Scoliosis
Ankylosing spondylitis

27
Q

Neuromuscular diseases which can cause restrictive lung disease

A

Polio
Guillain-Barre syndrome
Myasthenia gravis
Muscular dystrophy