Respiratory: IPF

Question 1

Describe what is meant by interstitial lung disease

Answer 1

Interstitial lung disease includes many conditions that cause inflammation and fibrosis of the lung parenchyma.

Question 2

Describe the symptoms of IPF [3]

Answer 2

• Dyspnoea
• Non-productive cough
• Fatigue

Question 3

Describe the signs of IPF [2]

Answer 3

Bibasal fine end-inspiratory crepitations
Clubbing

TOM TIP: Remember clubbing and bibasal fine inspiratory crackles in idiopathic pulmonary fibrosis. These patients are ideal for OSCEs as they are stable and have good signs.

Question 4

Describe what imaging results would indicate IPF? [2]

Answer 4

CXR:
- basilar, peripheral, bilateral, asymmetrical, reticular opacities (shadowing of the lung peripheries)

High-resolution CT scan (HRCT) of the thorax - definitive imaging
- “ground glass” in early stages
- honeycombing in later stages

Diffuse ground glass opacification

Question 5

Describe where the fibrosis is in this CXR [1]

Answer 5

Pulmonary fibrosis causes reticular (net-like) shadowing of the lung peripheries which is typically more prominent towards the lung bases

Question 6

Describe the spirometry results seen in IPF

Answer 6

Display a restrictive pattern:
- FEV1: decreased
- FVC decreased
(FEV1 and FVC are equally reduced)
- FEV1:FVC ratio greater than 70%

Question 7

Which antibodies are present in 30% of patients in IPF? [1]

Which other compound might be found? [1]

Answer 7

Anti-nuclear antibodies - ANA

Rheumatoid factor is found in 10%

Question 8

What does this CT depict in IPF? [1]

Answer 8

Honeycombing (advanced IPF)

Question 9

What would be the next step when investigating IPF if history, clinical evaluation, and high-resolution CT findings do not support a clear diagnosis of IPF? [1]

Answer 9

Surgical lung biopsy (via VAT): fibrosis of varying ages; areas of normal lung next to areas of honeycombing

Samples from two distinct areas are required

Question 10

How do you manage an acute axacerbation of IPF? [2]

Answer 10

1. High dose oral prednisolone
2. Condiser: cytotoxic therapy

BMJ BP

Question 11

How do you manage a patient not currently experiencing an acute exacerbation of IPF? [3]

Answer 11

1. Pirfenidone: anti-fibronolytic therapy
   OR
   Nintedanib
2. Smoking cessation; pulmonary rehab & oxygen
3. Consider: PPI if suffering from GORD symptoms: lomeprazole
4. Patients experiencing progressive deterioration: lung transplant

BMJ BP

Question 12

Explain the MoA of nintedanib? [1]

Answer 12

Nintedanib reduces fibrosis and inflammation by inhibiting tyrosine kinase

Question 13

Name 4 therapeutic drugs that are a risk factor for causing PF [4]

Answer 13

Amiodarone (also causes grey/blue skin)
Cyclophosphamide
Methotrexate
Nitrofurantoin

Question 14

Name 5 diseases that PF can occur secondary to [5]

Answer 14

Alpha-1 antitrypsin deficiency
Rheumatoid arthritis
Systemic lupus erythematosus (SLE)
Systemic sclerosis
Sarcoidosis

Question 15

What is the prognosis of IPF? [1]

Answer 15

2-5 years life expectancy from diagnosis.

Question 16

Which pathologies cause lung fibrosis in the upper [5] compared to lower zones [4]

Important to be able to ditinguish

Answer 16

Upper zones:
- hypersensitivity pneumonitis (also known as extrinsic allergic alveolitis)
- sarcoidosis
- TB
- Radiation-induced PF (post-breast / lung cancer; 12 months after)
- Coal workers penuomoconiosis

Lower zone:
- IPF
- Connective tissue disorders: e.g. SLE
- Drug induced: amiodarone, bleomycin, methotrexate
- Asbestosis

Question 17

What is an acronym for causes of upper zone fibrosis? [5]

Answer 17

C - Coal worker’s pneumoconiosis
H - Histiocytosis/ hypersensitivity pneumonitis
A - Ankylosing spondylitis
R - Radiation
T - Tuberculosis
S - Silicosis/sarcoidosis

Question 18

What is an acronym for causes of lower zone fibrosis? [4]

Answer 18

Lower (ACID)
- Asbestosis
- Connective tissue disorders (excluding Ank Spon eg. SLE)
- Idiopathic
- Drug induced (eg. Amiodarone, Bleomycin, Methotrexate)

Question 19

Describe what is known as hypersensitivity pneumonitis [2]

Answer 19

Type III and type IV hypersensitivity reaction to an environmental allergen

Inhalation of allergens in patients sensitised to that allergen causes an immune response, leading to inflammation and damage to the lung tissue.

Question 21

Name two drugs that can cause pulmonary fibrosis? [2]

Answer 21

Bleomycin & azathioprine

Question 22

Which of the following medications could be responsible for causing the gentleman’s pulmonary fibrosis?

Aspirin

Ramipril

Bleomycin

Spironolactone

Simvastatin

Answer 22

Bleomycin

Question 23

wWhich drug is a risk factor for causing pulmonary fibrosis? [1]

Answer 23

Methotrexate

Question 24

What is a pneumonic for remembering causes of pulmonary fibrosis? [5]

Answer 24

CHARTS

Coal-worker pneumoconiosis
Histiocytosis/hypersensitivity pneumonitis
Ankylosing spondylitis / RA - Methotrexate use
Radiation
Tuberculosis
Silicosis/sarcoidosis

Question 25

Asbesosis and Coal workers’ pneumoconiosis both cause interstitial lung fibrosis.

Where do each of them cause fibrosis in the lungs? [2]

Answer 25

Asbestos:
- lower lobes: in the roof and falls down

Silicosis and Coal workers’ pneumoconiosis:
- upper lobes drifts up from the ground to up