Idiopathic Pulmonary Fibrosis

Question 1

Idiopathic Pulmonary Fibrosis

Answer 1

Inflammatory cell infiltrate and pulmonary fibrosis of unknown cause

Question 2

Pathology

Answer 2

Usual interstitial pneumonia pattern (UIP)

Question 3

Usual Interstitial Pneumonia Pattern (UIP)

Answer 3

Heterogenous fibrosis in alveolar walls with fibroblastic foci & destruction of architecture causing honeycombing. Inflammation is minimal.

Question 4

Cause

Answer 4

Unknown

Question 5

Risk Factors

Answer 5

Smoking, drugs, rheumatoid arthiritis, SLE, systematic sclerosis, asbestos.

Question 6

Symptoms

Answer 6

Progressive dyspnoea, dry cough, malaise, weight loss, arthralgia, fatigue.

Question 7

Signs

Answer 7

Cyanosis, finger clubbing, bibasal fine end inspiratory crackles.

Question 8

Investigations

Answer 8

CXR, Spirometry, ABG, DCLO, HRCT, Lung Biopsy.

Question 9

CXR

Answer 9

Bilateral infiltrates, lower zone fibrosis

Question 10

Spirometry

Answer 10

Restrictive defect on PFTs, reduced FEV1 & FVC with normal or raised FER. Reduced lung volumes & low gas transfer.

Question 11

ABG

Answer 11

Reduced PaO2, increased PaCO2

Question 12

DCLO

Answer 12

Reduced

Question 13

HRCT

Answer 13

Honeycombing cystic changes, traction bronchiectasis, reticulonodular fibrotic shadowing that is worse at lung bases & periphery.

Question 14

Complications of IPF

Answer 14

Resp failure & increased risk of lung cancer

Question 15

General Treatment

Answer 15

Smoking cessation, pulmonary rehab, oxygen if hypoxic.

Question 16

Permanent treatment in young patients

Answer 16

Lung transplant

Question 17

5 year mortality

Answer 17

50% will die within 5 years

Question 18

Drug treatment

Answer 18

Antifibrotic drugs (pirfenidone & nintedanib) slows progression.