Idiopathic Pulmonary Fibrosis

Question 1

What is Idiopathic Pulmonary Fibrosis (IPF)?

Answer 1

Idiopathic pulmonary fibrosis (IPF) is a rare, chronic, life-threatening disease that manifests over several years and is characterised by the formation of scar tissue within the lungs and progressive dyspnoea.
IT IS AN INTERSTITIAL LUNG DISEASE.

Question 2

What are the risk factors of IPF?

Answer 2

Age
Male
FHx
Smokers (due to oxidative injury)
GORD/ Dust/ infection/ diabetes

Question 3

What is the epidemiology of IPF?

Answer 3

The precise incidence and prevalence of IPF are not known. The prevalence in the US has been estimated to be between 14.0 and 42.7 cases per 100,000 people. Similar results have been reported in Finland and in the UK.

Question 4

What are the signs and symptoms of IPF?

Answer 4

Dyspnoea
Cough
Weight loss, fatigue, malaise
Crackles
Clubbing

Question 5

What investigations are important for IPF?

Answer 5

CXR
Basilar, peripheral, bilateral, asymmetrical, reticular opacities

High resolution CT
Basilar and subpleural predominat areas of increased reticultion (honeycombing)
Traction bronchiectasis

Anti-nuclear antibodies
Normal or elevated

Rheumatoid factor
Normal or elevated

Anti cyclic citrullinated peptide
Normal

Myositis panel
Normal