Interstitial Lung Disease/Pulmonary Fibrosis Flashcards

1
Q

What is the interstitium?

A

Tissue that lies b/w the alveoli & pulmonary capillaries

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2
Q

What is interstitial lung disease?

A

A number of conditions diffusely affecting the lung parenchyma

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3
Q

What are the common features of interstitial lung disease?

A

Repeated exogenous/endogenous stimuli
Chronic inflammation
Fibrosis of the interstitium
Restrictive lung disease

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4
Q

What are the causes of interstitial lung disease?

A

2o to exogenous stimuli
2o to endogenous stimuli (systemic disorders)
Idiopathic

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5
Q

What exogenous stimuli commonly cause interstitial lung disease?

A

Occupational/Environmental - Asbestosis, silicosis, coal dust
Drugs - Nitrofurantoin, amiodarone, sulfasalazine, methotrexate
Hypersensitivity reactions - Extrinsic Allergic Alveolitis
Infections - TB, fungal/viral infections

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6
Q

What endogenous stimuli commonly cause interstitial lung disease?

A

Sarcoidosis
Rheumatoid arthritis
CTDs - SLE, systemic sclerosis, Sjogren’s

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7
Q

What is the main idiopathic cause of interstitial lung disease?

A

Idiopathic pulmonary fibrosis

-most common cause of interstitial lung disease

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8
Q

What are the classical sx of interstitial lung disease?

A

Dyspnoea on exertion

Non-productive ‘dry’ cough

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9
Q

What are the common signs of interstitial lung disease?

A
Clubbing
Respiratory distress
Reduced expansion
Normal unless 2o pathology
Fine end inspiratory crackles +/- exp wheeze
Pulmonary HTN, RHF
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10
Q

What is the aetiology of idiopathic pulmonary fibrosis?

A

Cryptogenic fibrosing alveolitis

Onset in late 60s, males

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11
Q

What are the risk factors for idiopathic pulmonary fibrosis?

A

Smoking
Chronic aspiration
Antidepressants
Infections

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12
Q

What are the common sx of idiopathic pulmonary fibrosis?

A
Dry cough
Exertional dyspnoea
Malaise
Wt loss
Arthralgia
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13
Q

What are the common signs of idiopathic pulmonary fibrosis?

A

Fine end-inspiratory crackles
Cyanosis
Finger clubbing

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14
Q

What are the common complications of idiopathic pulmonary fibrosis?

A

Type 2 resp failure
Pulmonary HTN
Lung cancer

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15
Q

What investigations are appropriate in suspected interstitial lung disease?

A
FBC, ANA
CXR
High-res CT (honeycombing)
Spirometry (restrictive deficit, reduced gas exchange)
Bronchoalveolar lavage
Lung biopsy
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16
Q

What signs of interstitial lung disease can be seen on CXR?

A

Reduced lung volume

Bilaterial reticulo-nodular shadowing

17
Q

What are the management options of interstitial lung disease?

A

Often unresponsive to treatment
20% respond to long courses of prednisolone
Lung transplant