Restrictive lung disease

Question 1

What are some causes of restriction of the thoracic cavity due to disease outwith the lung?

Answer 1

Obesity
Musculoskeletal e.g. kyphoscoliosis, ankylosing spondylitis
Neurological e.g. motor neuron disease, Guillian-barre syndrome, myasthenia gravis
Ascites

Question 2

What is the broad term for restrictive lung disease where pathology within the lung is causing restriction?

Answer 2

Diffuse parenchymal lung disease (DPLD)

Question 3

What is the pathophysiology of DPLD?

Answer 3

Disease occurs in alveolar walls or lumen

Impairs oxygen exchange between alveoles and ateries

Question 4

What broad pathologies might cause DPLD?

Answer 4

Fluid in the alveolar air spaces
Consolidation in the alveolar air spaces
Inflammatory infiltration of the alveolar walls

Question 5

What causes fluid to build up in the alveolar spaces?

Answer 5

Cardiac or non-cardiac pulmonary oedema

Question 6

What is the difference between cardiac and non cardiac pulmonary oedema in terms of venous pressures?

Answer 6

Cardiac effusions are due to high venous pressures as a result of cardiac pathology e.g. left ventricular failure
Non-cardiac effusions will have normal venous pressures but leaky capillaries causing oedema e.g. due to sepsis, trauma or altitude sickness

Question 7

What might cause consolidation in the alveolar air spaces?

Answer 7

Infective pneumonia

Infarction

Question 8

What types of inflammatory infiltration may affect the alveolar walls?

Answer 8

Granulomatous 
Drug induced
Toxic gas/fumes
Fibrosis
Autoimmune
Dust disease
Carcinoma

Question 9

What examples of granulomatous alveolitis are there?

Answer 9

Extrinsic allergic alveolitis, type 3 hypersensitivity e.g. farmers lung
Sarcoidosis

Question 10

What drugs may cause DPLD?

Answer 10

Amiodarone
Methotrexate
Gold

Question 11

What conditions may cause fibrosing alveolitis?

Answer 11

Rheumatoid disease

Question 12

What conditions may cause autoimmune alveolitis?

Answer 12

SLE
Wegener’s granulomatosis
Polyarteritis

Question 13

What are the two broad categories of dust disease?

Answer 13

Fibrogenic and non-fibrogenic

Question 14

What are examples of fibrogenic dust disease?

Answer 14

Asbestosis and silicosis

Question 15

What is the clinical presentation of DPLD?

Answer 15

Cough but no wheeze
Breathlessness on exertion
Finger clubbing
Inspiratory crackles
Cyanosis

Question 16

What does spirometry show in restrictive lung disease?

Answer 16

Reduced FEV1, reduced FVC, normal FEV1/FVC ratio

Question 17

What is the first line treatment of DPLD?

Answer 17

Systemic corticosteroids e.g. oral prednisolone

Question 18

What is the second line treatment of DPLD?

Answer 18

Azathioprine