Idiopathic pulmonary fibrosis Flashcards Preview

Year 3: respiratory conditions > Idiopathic pulmonary fibrosis > Flashcards

Flashcards in Idiopathic pulmonary fibrosis Deck (10)
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1
Q

define idiopathic pulmonary fibrosis?

A

inflammatory condition of the lung resulting in progressive fibrosis of the alveoli and interstitium

2
Q

what is the pathophysiology of this condition?

A
  • Recurrent injury to alveolar epithelial cells results in secretion of cytokines and growth factors
  • This leads to fibroblast activation, recruitment, proliferation, differentiations into myofibroblasts and increased collagen synthesis and deposition
3
Q

risk factors of idiopathic pulmonary fibrosis?

A

o Smoking
o Occupational exposure to metal or wood dust
o Chronic microaspiration
o Animal and vegetable dusts

4
Q

what is the epidemiology of pulmonary fibrosis?

A
  • More common in MALES

* Ages 50-70

5
Q

presenting symptoms of idiopathic pulmonary fibrosis?

A
  • Gradual-onset, progressive dyspnoea on exertion
  • Dry irritating cough
  • Bi-basal fine end inspiratory creps
  • Fatigue and weight loss are common
6
Q

signs of idiopathic pulmonary fibrosis?

A
  • Clubbing (50%)

* Bibasal fine late inspiratory crackles

7
Q

what might the bloods show?

A

o ABG
• Normal in early disease
• PO2 decreases with exercise
• Normal PCO2, which rises in late stage disease
o ANA and Rheumatoid Factor
• 1/3 of patients are positive for ANA, 1/10 positive for RF

8
Q

what might a chest xray show?

A
  • Early disease may show bilateral ground glass shadowing

- late disease eventually, honeycombing

9
Q

what is the investigation of choice?

A

high res CT

10
Q

what might the lung function tests show?

A

Restrictive features:
o reduced FEV1 and FVC
o with preserved or increased FEV1/FVC
o Impaired gas exchange (reduced TLCO)