Interstitial lung disease Flashcards

1
Q

How do patients with interstitial lung disease often present ?

A

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

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2
Q

What do lung function tests usually show

A

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

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3
Q

What is a classic example of a diffuse fibrotic lung disease

A

Idopathic pulmonary fibrosis

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4
Q

Does Iditiopathiic pulmonary fibrosis usually affect men or women

A

Men: 2:1

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5
Q

Do patients with Idiopathic pulmonary fibrosis have clubbing

A

Yes (60-70% do)

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6
Q

What causes idiopathic pulmonary fibrosis

A

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

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7
Q

What do lung biospies of idiopathic pulmonary fibrosis show

A

usual interstitial pneumonia

Honeycombing

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8
Q

Where are the changes more severe

A

Subpleurally

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9
Q

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

A

High resolution CT

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10
Q

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

A

Ground Glass opacities

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11
Q

What is the treatement for idiopathic pulmonary fibrosis

A

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

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12
Q

What is the success rate of treatment of idiopathic pulmonary fibrosis

A

50% mortality within 3 years

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13
Q

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

A

Lung transplantation

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14
Q

What causes idiopathic pulmonary fibrosis

A

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

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15
Q

What is the treatment for idiopathic pulmonary fibrosis

A

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

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16
Q

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

A

Lung transplantation

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17
Q

What drug can cause cryptogenic organising pneumonia

A

Amiodarone

18
Q

What do patients clinically present with

A

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

19
Q

What might a patient be mistakenly diagnosed with

A

infective Pneumonia

20
Q

What might a patient be mistakenly diagnosed with

A

infective Pneumonia

21
Q

What does cryptogenic organising pneumonia respond to

A

Corticosteriods

22
Q

Idiopathic pulmonary fibrosis is commonly associated with which other disease

A

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

23
Q

What is Caplan’s syndrome

A

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

24
Q

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

A

Drugs used to treat rheumatoid disease

25
Q

What might pleural effusions cause

A

Pleural thickening

26
Q

What is extrinsic allergic alveolitis

A

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

27
Q

What are 2 examples of extrinsic allergic alveolitis

A

Farmer’s lung and bird fancier’s lung

28
Q

What type of organism give rise to extrinsic allergic alveolitis

A

Fungi

29
Q

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

A

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

30
Q

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

A

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

31
Q

What is the chronic form of extrinsic allergic alveolitis characterised by

A

Insidious development of dyspnoea and lung fibrosis

32
Q

What is the main treatment of extrinsic allergic alveolitis?

A

Removal of the antigen - cessation of exposure

33
Q

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

A

Steroids (prednisolone 40mg/day)

34
Q

What is sarcoidosis

A

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

35
Q

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

A

CD4 lymphocytes

36
Q

What are the serum immunoglobulin levels like in acute sarcoidosis

A

Usually elevated

37
Q

Describe the clinical findings in acute sarcoidosis

A
Erythema nodosum (Round red raised nodules typically over the shins) and bilateral hilar lymphadenopathy 
Sometimes uveitis, arthritis and parotits
38
Q

Describe the clinical appearance of chronic sarcoidosis

A

Reticular shadowing often distributed in a perihilar fashion of CXR

39
Q

Describe the appearance of chronic pulmonary sarcoidosis

A

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

40
Q

Describe the appearance of chronic pulmonary sarcoidosis

A

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

41
Q

How is sarcoidosis diagnosed

A

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

42
Q

How is sarcoidosis treated

A

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