Interstitial lung disease

Question 1

How do patients with interstitial lung disease often present ?

Answer 1

With progressive breathlessness,
a dry cough,
lung crackles
Diffuse infiltrates on CXR

Question 2

What do lung function tests usually show

Answer 2

A restrictive pattern
–> normal ratio but decreased VC
An impaired gas diffusion
Hypoxaemia with hypocapnia

Question 3

What is a classic example of a diffuse fibrotic lung disease

Answer 3

Idopathic pulmonary fibrosis

Question 4

Does Iditiopathiic pulmonary fibrosis usually affect men or women

Answer 4

Men: 2:1

Question 5

Do patients with Idiopathic pulmonary fibrosis have clubbing

Answer 5

Yes (60-70% do)

Question 6

What causes idiopathic pulmonary fibrosis

Answer 6

Not entirely undertood but thought to be the result of a ffailyre of repair of lung tissuem whereby epithelial injury culminates in fibrosis rather than a controlled inflammatory and healing process

Question 7

What do lung biospies of idiopathic pulmonary fibrosis show

Answer 7

usual interstitial pneumonia

Honeycombing

Question 8

Where are the changes more severe

Answer 8

Subpleurally

Question 9

What can show evidence of advanced fibrosis with extensive areas of reticulation and honeycombing

Answer 9

High resolution CT

Question 10

What is a key feature of idiopathic pulmonary fibrosis on an Xray

Answer 10

Ground Glass opacities

Question 11

What is the treatement for idiopathic pulmonary fibrosis

Answer 11

A combination of low-dose prednisolone, azathiprine and N-acetyl cysteine

Question 12

What is the success rate of treatment of idiopathic pulmonary fibrosis

Answer 12

50% mortality within 3 years

Question 13

What might be a treatment option for younger patients with idiopathic pulmonary fibrosis

Answer 13

Lung transplantation

Question 14

What causes idiopathic pulmonary fibrosis

Answer 14

Not entirely understood but thought to be the result of a failure of repair of lung tissue whereby epithelial injury culminates in fibrosis rather than a controlled inflammatory and healing process

Question 15

What is the treatment for idiopathic pulmonary fibrosis

Answer 15

A combination of low-dose prednisolone, azathiprine and N-acetyl cysteine

Question 16

What might be a treatment option for younger patients with idiopathic pulmonary fibrosis

Answer 16

Lung transplantation

Question 17

What drug can cause cryptogenic organising pneumonia

Answer 17

Amiodarone

Question 18

What do patients clinically present with

Answer 18

Cough, malaise, fever, dyspnoea with chest Xray infiltrates and an elevated erythrocyte sedimentation rate

Question 19

What might a patient be mistakenly diagnosed with

Answer 19

infective Pneumonia

Question 20

What might a patient be mistakenly diagnosed with

Answer 20

infective Pneumonia

Question 21

What does cryptogenic organising pneumonia respond to

Answer 21

Corticosteriods

Question 22

Idiopathic pulmonary fibrosis is commonly associated with which other disease

Answer 22

Connective tissue disease - Raynaud’s phenomenon, inflammatory arthritis, sicca syndrome, myositis and skin changes

Question 23

What is Caplan’s syndrome

Answer 23

When rheumatoid disease occurs in association with coalworker’s pneumoconiosis and large cavitating nodules may develop

Question 24

What may give rise to inflammatory reactions within the lung and lung infection

Answer 24

Drugs used to treat rheumatoid disease

Question 25

What might pleural effusions cause

Answer 25

Pleural thickening

Question 26

What is extrinsic allergic alveolitis

Answer 26

An immunologically mediated lung disease in which a hypersenstivity response occurs in a sensitised individual to an inhaled antigen

Question 27

What are 2 examples of extrinsic allergic alveolitis

Answer 27

Farmer's lung and bird fancier's lung

Question 28

What type of organism give rise to extrinsic allergic alveolitis

Answer 28

Fungi

Question 29

In the acute form of extrinsic allergic alveolitis, what does the patient experience

Answer 29

Recurrent episodes of dyspnoea, dry cough, pyrexia, myalgia and a flu like sensation which occurs about 4-8 hours after antigen exposure

Question 30

In the acute form of extrinsic allergic alveolitis, what does the patient experience

Answer 30

Recurrent episodes of dyspnoea, dry cough, pyrexia, myalgia and a flu like sensation which occurs about 4-8 hours after antigen exposure

Question 31

What is the chronic form of extrinsic allergic alveolitis characterised by

Answer 31

Insidious development of dyspnoea and lung fibrosis

Question 32

What is the main treatment of extrinsic allergic alveolitis?

Answer 32

Removal of the antigen - cessation of exposure

Question 33

What can be used during a severe acute episode of extrinsic allergic alveolitis

Answer 33

Steroids (prednisolone 40mg/day)

Question 34

What is sarcoidosis

Answer 34

A multisystem disease characterised by the occurrence in affected organs of non-caseating granulomatous lesions that may progress to cause fibrosis

Question 35

Accumulation of what at disease sites is suggestive of an immunological reaction to an unidentified poorly degradable antigen

Answer 35

CD4 lymphocytes

Question 36

What are the serum immunoglobulin levels like in acute sarcoidosis

Answer 36

Usually elevated

Question 37

Describe the clinical findings in acute sarcoidosis

Answer 37

``` Erythema nodosum (Round red raised nodules typically over the shins) and bilateral hilar lymphadenopathy Sometimes uveitis, arthritis and parotits ```

Question 38

Describe the clinical appearance of chronic sarcoidosis

Answer 38

Reticular shadowing often distributed in a perihilar fashion of CXR

Question 39

Describe the appearance of chronic pulmonary sarcoidosis

Answer 39

This involves the lung parenchyma with reticular shadowing often distributed in a perihilar fashion on CXR Some patients may have progressive fibrosis and loss of lung function with impairment of fas diffusion, reduction in lung volumes and sometimes airways obstruction with air trapping and bulla formation

Question 40

Describe the appearance of chronic pulmonary sarcoidosis

Answer 40

This involves the lung parenchyma with reticular shadowing often distributed in a perihilar fashion on CXR Some patients may have progressive fibrosis and loss of lung function with impairment of fas diffusion, reduction in lung volumes and sometimes airways obstruction with air trapping and bulla formation

Question 41

How is sarcoidosis diagnosed

Answer 41

Based on clinical grounds especially with classical features such as erythema nodosum and BHL

Question 42

How is sarcoidosis treated

Answer 42

It is a self limiting disease that resolves spontaneously without treatment usually. Minority require corticosteroids due to the progressive fibrosis