Idiopathic Pulmonary Fibrosis

Question 1

What is IPF?

Answer 1

The most common ILD
Progressive interstitial fibrosis of unkown aetiology
Most common in men50+

Question 2

What happens during IPF?

Answer 2

Patchy interstitial chronic inflammation (mostly in lower zones) with resultant:
- Type 2 pneumocyte hyperplasia
- SM & vascular proliferation
- Fibroblastic Foci proliferation
Leading to scarred & fibrosed lung possibly with honeycombing cysts

Question 3

How does IPF present?

Answer 3

Progressive:
Dyspnoea
Couhg
Basal Crackles
Cyanosis
Clubbing
Malaise
Weight Loss
Arthralgia

Question 4

How do we investigate IPF?

Answer 4

PFT:

• Restrictive Pattern
• Lower Gas Transfer

ABG - CRP

Bronchoalveolar Lavage:

• Lymphocytes
• Eosinophils
• Neutrophils

Biopsy (histological exam)

CT

CXR:

• Diffuse infiltrates
• Patchy Fibrosis
• Cysts (ground glass appearance)
• May have lower lung volume (lower compliance and expansion)
• Temporal or spatial heterogenicity as it doesnt develop evenly over lung

Question 5

How do we treat IPF?

Answer 5

Best supportive care as there is no cure:
Anti-Fibrotic Agents - Pirfenidone
Analgesia
Lung Transplant
LTOT
Pulm. Rehab