FN:Idiopathic Pulmonary Fibrosis (CFA)

Question 1

Epi

Answer 1

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

Question 2

Presentation Symptoms

Answer 2

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

Question 3

Presentation Signs

Answer 3

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

Question 4

Complications

Answer 4

􏰀 ↑ risk Ca lung

􏰀 Type 2 respiratory failure and cor pulmonale

Question 5

Ix bloods

Answer 5

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

Question 6

Ix CXR

Answer 6

􏰀 ↓ lung volume

􏰀 Bilat lower zone retic-nod shadowing 􏰀 Honeycomb lung

Question 7

Ix HRCT

Answer 7

􏰀 Shows similar changes to CXR 􏰀 More sensitive

Question 8

Ix Spiro shows

Answer 8

􏰀 Restrictive defect 􏰀 ↓ transfer factor

Question 9

Ix Other

Answer 9

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

Question 10

Mx

Answer 10

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

Question 11

Prognosis

Answer 11

􏰀 50% 5yr survival