Pulmonary Fibrosis

Question 1

What is pulmonary fibrosis?

Answer 1

Restrictive disease caused by inflammation and scarring of the lungs
A progressive condition where the scarring and inflammation build up, this build up is called fibrosis
Can be known as cryptogenic fibrosing alveoli this or usual interstitial pneumonia
Resulting in reduced gas exchange
Scarring will not recover, therefore progressive worsening

Question 2

Causes of pulmonary fibrosis and who’s most likely to be effected -

Answer 2

Men are more likely to have it
And more likely to affect older people
Cause is unknown - IPF - idiopathic pulmonary fibrosis

More common in people who smoke or have smoked
?occupational exposure - dust from wood, metal, textile or stone
?infection and some viruses such as herpes and hep C
Some link to people with GORD (gastroc-oesophageal reflux disease) which causes heart burn - link with this leading to lung scarring
Few cases - genetic link, but not sure why!

Question 3

Symptoms of pulmonary fibrosis -

Answer 3

Main symptom is breathlessness, very severely in some cases
Some people symptoms gradually progress, others they very quickly get worse
Constant cough that doesn’t go away
Feeling tired all the time
Clubbing of fingers and toes
It is NOT infectious = cannot pass it on

Question 4

Is there a cure?

Answer 4

No, almost half of people with IPF will not live for more than 3 years after their diagnosis

Question 5

How do you diagnose PF?

Answer 5

Detailed patient history
Clinical signs - breathlessness at rest and on exertion
Cough that is persistent
Clubbing

Spirometry - looking for restrictive pattern here
Bronchoscopy - check airways and look for abnormalities in the lung tissue
CXR followed by a CT scan

Question 6

Treatment -

Answer 6

O2 therapy
Pulmonary rehab to manage breathlessness and maintain and improve exercise tolerance
Medications - sedatives and morphine to help with breathlessness
Treatment for heart burn as it makes you feel breathless, and a blocked nose
Lung transplant - very rare! You need to be fit and will enough by the time a match becomes available

Question 7

Medications
Pirfenidone -
Nintedanib -

+ their side effects -

Answer 7

Pir - used in mild to moderate IPF. Capsule that is thought to slow down the inflammation and build up of scar tissue
Side effects - sickness, diarrhoea, skin more reactive to sunlight

Nin - originally for lung cancer patients
Also reduces rate at which lungs become scarred and helps reduce ‘flare ups’
Only perscribed to patients who have forced vital capacity between 50-80% of their predicted
Treatment will be stopped if process continues to progress
Side effects - abdominal pain, potential haemorrhage, diarrhoea and weight loss

Question 8

Pulmonary rehab, why?

Answer 8

Exercise - to help them be as efficient with o2 usage
Education
Self management
Diet - eating is a challenge due to breathlessness
Lifestyle modifications

Will help with management of breathlessness and exercise tolerance

Question 9

How can patients help themselves?

Answer 9

Stop smoking
Have early flu jabs
Have pneumococcal vaccination
Avoid people with chest infections and colds
Stay as fit and active as possible
Eat healthy balanced diet
Get in touch with British lung foundation for further support

Question 10

What does interstitial lung tissue mean?

Answer 10

Means between and around the alveoli
This is where PF occurs

Question 11

What would you hear on auscultation?

Answer 11

Reduced breath sounds
Fine crackles on end of inspiration

Question 12

What would you see on a CXR?

What would spirometry results look like?

Answer 12

Honeycombing
Normal FEV1/FVC ratio and reduced FVC