Pulmonary fibrosis Flashcards

1
Q

What is the definition of pulmonary fibrosis?

A

Chronic interstitial lung damage and loss of elasticity of the lungs

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2
Q

How common is pulmonary fibrosis?

A

Common, with incidence of around 7.44 per 100,000 population of the UK

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3
Q

What is the pathology of pulmonary fibrosis?

A
  • Remodelling of the interstitium/replacement of damaged lung tissue
  • Chronic inflammation
  • Hyperplasia of type II epithelial cells/type II pneumocytes
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4
Q

What are the risk factors/aetiology of pulmonary fibrosis?

A
  • Known cause:
  • Occupational/environmental e.g. asbestos, silicosis, cotton workers lung
  • Drugs e.g. nitrofurantoin, amiodarone, sulfasalazine
  • Hypersensitivity reactions
  • Infections e.g. TB, fungi, viral
  • GORD
  • Associated with systemic disorders:
  • Sarcoidosis
  • RA
  • SLE, Sjorgren’s syndrome
  • UC, renal tubular acidosis, autoimmune thyroid disease
  • Idiopathic
  • ↑ age
  • Smoking
  • COPD
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5
Q

What are the signs/symptoms of pulmonary fibrosis?

A
  • Dyspnoea on exertion
  • Non productive paroxysmal cough
  • Bilateral fine inspiratory crackles on auscultation
  • Clubbing
  • Abnormal CXR/CT
  • Restrictive pulmonary spirometry – diffusing capacity of the lung for carbon monoxide
  • Flu like symptoms, fatigue, weight loss
  • Arthalgia, muscle pains, skin rashes
  • Obstructive sleep apnoea
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6
Q

What investigations are carried out for suspected pulmonary fibrosis?

A
  • FBC, ESR, CRP, autoantibodies
  • CXR
  • Lung function test – spirometry
  • High resolution CT of thorax
  • Transbonchial/surgical lung biopsy
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7
Q

What are the surgical treatments for pulmonary fibrosis?

A

Lung transplant

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8
Q

What are the pharmacological treatments for pulmonary fibrosis?

A
  • Oxygen
  • Influenza and pneumococcal vaccinations
  • PPI
  • Opiates – in end stage
  • Corticosteroids – acute exacerbation
  • Pirfenidone – in FVC is between 50-80%
  • Nitendanib
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9
Q

What are the non pharmacological treatments for pulmonary fibrosis?

A
  • Pulmonary rehabilitation
  • Physiotherapy
  • Regular exercise to control weight
  • Smoking cessation
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