Condition- Idiopathic Pulmonary Fibrosis

Question 1

What is idiopathic pulmonary fibrosis?

Answer 1

It is a chronic lung condition characterised by progressive fibrosis of he interstitium of the lung. Idiopathic= No known cause

Question 2

Which gender is IPF more common in?

Answer 2

Males

Question 3

What sort of lung disease is IPF?

Answer 3

Restrictive Lung Disease

Question 4

List some of the symptoms of IPF…

Answer 4

• Progressive exertional dyspnoea
• Dry cough
• Fatigue + weight loss may also feature
• Important to take drug and occupational hx

Question 5

List some of the signs of IPF on clinical examination:

(Think about 1 in hands and what can be heard on auscultation)

Answer 5

• clubbing (50%)
• cyanosis
• Bibasal find end-insipiratory crackles
• Signs of RHF

Question 6

List some important investigations to conduct on someone with suspected IPF…

Answer 6

1. Spirometry
2. CXR
3. High res CT
4. Bloods

Question 7

Which autoantibodies might be elevated in the bloods of someone with IPF?

Answer 7

• ANA
• Rheumatoid Factor

Seen in a 1/3rd of patients

Question 8

What might the spirometry results of someone with IPF be?

Answer 8

• FEV1 normal/ decreased
• FVC decreased
• Increased or normal FEV1/FEVC ratio (restrictive)
• All lung volumes and capacities decreased

Question 9

What might be seen in a CXR of a patient with IPF?

Answer 9

Bilateral Interstitial Shadowing= small, irregular, peripheral opacities

‘ground glass’ progressing to ‘honeycombing’- this is caused by thickening of the walls of the alveoli (intersitium) in a honey comb pattern

But CT needed to make diagnosis of IPF

Question 10

Injury to the alveolar lining can cause Type 1 pneumocytes to release a mediator which stimulate Type 2 pneumocytes to stimulate proliferation and differentiation of myofibroblasts- what is the name of this mediator?

Answer 10

transforming growth factorbeta1 (TGFB1)

Question 11

List three main risk factors for the development of IPF

Answer 11

Old Age

Male

Smoker

Question 12

What sort of pattern can be seen in this CT chest of a patient with IPF?

Answer 12

Honeycombing

Question 13

List 3 drugs which are associated with IPF

Answer 13

• methotrexate
• amiodarone
• bleomycin

Question 14

Which investigation is necessary to make a diagnosis of IPF?

Answer 14

CT

Question 15

Which part of the lungs does IPF normally affect?

Answer 15

The lower zones