FN:Idiopathic Pulmonary Fibrosis (CFA) Flashcards Preview

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Flashcards in FN:Idiopathic Pulmonary Fibrosis (CFA) Deck (11):
1

Epi

􏰀 Commonest cause of interstitial lung disease
􏰀 Middle age
􏰀 M>F = 2:1
􏰀 Assoc. ̄c other AI disease in 30%

2

Presentation Symptoms

􏰀 Dry cough
􏰀 Dyspnoea
􏰀 Malaise, wt. loss 􏰀 Arthralgia
􏰀 OSA

3

Presentation Signs

􏰀 Cyanosis
􏰀 Clubbing
􏰀 Crackles: fine, end-inspiratory

4

Complications

􏰀 ↑ risk Ca lung
􏰀 Type 2 respiratory failure and cor pulmonale

5

Ix bloods

􏰀 ↑CRP
􏰀 ↑Ig
􏰀 ANA+ (30%)
􏰀 RF+ (10%)
􏰀 ABG: ↓PaO2, ↑PaCO2

6

Ix CXR

􏰀 ↓ lung volume
􏰀 Bilat lower zone retic-nod shadowing 􏰀 Honeycomb lung

7

Ix HRCT

􏰀 Shows similar changes to CXR 􏰀 More sensitive

8

Ix Spiro shows

􏰀 Restrictive defect 􏰀 ↓ transfer factor

9

Ix Other

􏰀 Lung biopsy: Usual interstitial pneumonia 􏰀 BAL: may indicate disease activity
􏰁 ↑ lymphocytes: good prognosis
􏰁 ↑ PMN or eosinophils: bad prognosis 􏰀 DTPA scan: may reflect disease activity

10

Mx

􏰀 Supportive care
􏰁 Stop smoking
􏰁 Pulmonary rehabilitation
􏰁 O2 therapy
􏰁 Palliation
􏰁 Rx symptoms of heart failure
􏰀 Lung Tx offers only cure

11

Prognosis

􏰀 50% 5yr survival

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