Interstitial Lung Disease Flashcards

1
Q

What is interstitial lung disease

A

Conditions that affect lung parenchyma diffusely, characterised by progressive interstitial fibrosis + inflammation

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2
Q

Interstitial lung disease clinical features

A

Dyspnoea on exertion
Non-productive paroxysmal cough
Abnormal breath sounds + CXR/CT
Restrictive spirometry with decreased DLCO

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3
Q

Interstitial lung disease pathology

A

Fibrosis + remodelling of interstitium
Chronic inflammation
Hyperplasia of type II epithelial cells/pneumocytes

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4
Q

ILD classification

A

Known cause
Systemic disorders
Idiopathic

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5
Q

What is idiopathic pulmonary fibrosis

A

Commonest cause of ILD, inflammatory cell infiltrates + fibrosis of unknown cause

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6
Q

Idiopathic pulmonary fibrosis signs + symptoms

A

Dry cough
Exertional dyspnoea
Malaise, weight loss
Arthralgia

Cyanosis
Clubbing
Fine end-inspiratory crepitations

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7
Q

Idiopathic pulmonary fibrosis complications

A

Resp failure

Inc lung cancer risk

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8
Q

Idiopathic pulmonary fibrosis tests

A

Hypoxic, hypercapnic if severe on ABG

Inc CRP, ANA in some

CXR shows dec lung volume, bilateral lower zone reticulo-nodular shadows
CT shows honeycomb lung in advanced disease
Lung biopsy may be needed for diagnosis, usual interstitial pneumonia describes biopsy findings

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9
Q

Idiopathic pulmonary fibrosis management

A

Supportive care

Trials + transplant

In future:
Nintedanib (targets growth factors)
Pirfenidone (immunosuppressant and anti-fibrotic)

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10
Q

Idiopathic pulmonary fibrosis prognosis

A

50% 5yr survival

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