6 - lung fibrosis treatment

Question 1

methods to monitor IPF progression

Answer 1

pulmonary function tests

thoracic imaging

Question 2

example of types of pulmonary function tests

Answer 2

forced vital capacity 
diffusion capacity of the lungs 
forced expiratory volume (FEV)
oxyhaemoglobin saturation 
6-minute walk test

Question 3

method for thoracic imaging

Answer 3

high resolution CT scan

Question 4

only possible cure for IPF

Answer 4

lung transplant

Question 5

why is palliative care important for IPF

Answer 5

IPF is almost always a terminal disease

Question 6

methods to diagnose IPF

Answer 6

listening for crackles through stethoscope
thoracic imaging
lung function tests
BAL fluid

endoscopic lung biopsy

Question 7

results of 6 min walk test

Answer 7

often only 250m for IPF patients

should be around 700m

Question 8

treatment strategies for IPF

Answer 8

proton pump inhibitor (PPI)
anti-coagulants
pharmaceuticals
pulmonary rehabilitation

Question 9

example of proton pump inhibitor (PPI) to treat IPF

Answer 9

omeprazole

Question 10

aim of omeprazole

Answer 10

proton pump inhibitor

reduce stomach acid made in the stomach for reflux to reduce coughing

Question 11

types of pharmaceutical therapies used to treat IPF

Answer 11

tyrosine kinase inhibitor (nintedanib)

antifibrotic agent (pirfendione)

antiviral therapies (ganciclovir)

Question 12

nintedanib

Answer 12

RTK inhibitor
blocks downstream signalling by inhibitiing cytokine receptors
slows disease progression

Question 13

mechanism underlying nintedanib

Answer 13

if tyrosine kinase receptor is inhibited
cytokines (e.g. PDGF/VEGF/FGF) cannot act
therfore fibroblast proliferation is inhibited and inflammation is reduced
slows disease progression

Question 14

testing for the presence of viruses in IPF

Answer 14

molecular techniques such as PCR, qPCR

testing for presence of antibodies –> shows past exposure to virus

Question 15

why is therapy for IPF important

Answer 15

no realistic cure currently

main aim is to relieve symptoms and slow progression

Question 16

pirfenidone

Answer 16

slows development of scarring in the lungs by reducing the immune response

Question 17

N-acetylcysteine

Answer 17

slows progression of IPF
anti-oxidant

loosens thick mucous

Question 18

other potenetial targets for anti-fibrotic drugs

Answer 18

CYTOKINES (IL-3, IL-21), chemokines
angiogenic factors (VEGF)
growth fators
acute phase proteins
caspases

Question 19

non-pharmaceutical treatment methods

Answer 19

improve lifestyle
oxygen therapy
pulmonary rehabilitations
lung transplant

Question 20

methods to improve lifestyle

Answer 20

–> stop smoking, exercise regularly, eat healthily, have seasonal vaccinations

Question 21

pulmonary rehabilitation methods

Answer 21

physical exercises
breathing exercises e.g.singing
psychological and social support
education about IPF

Question 22

importance of oxygen therapy

Answer 22

blood oxygen decreases in IPF causing breathlessness

treatment allows patient to be more active

Question 23

method of oxygen therapy

Answer 23

via nasal tube or mask attached to machine

Question 24

current clinical trials for IPF treatment

Answer 24

one trial on TGF-beta 1
one on FG-3019 –> shows reduction in fibrosis in animal models
research into steroids to exert anti-inflammatory effects

Question 25

ganciclover

Answer 25

anti-viral therapy

shows promising effects when combined with standard therapy

Question 26

why are animal studies important

Answer 26

used to demonstrate how, during latency, herpesvirus infections reprogram lung epithelial cells to produce profibrotic factors making lungs more susceptible to fibrotic insult

Question 27

median survival of IPF patients

Answer 27

2-3 years

Question 28

common name for PPI drugs

Answer 28

antacids

Question 29

FGF

Answer 29

fibroblast growth factor

Question 30

effect of nintedinab action

Answer 30

fibroblast proliferation is inhibited and inflammation is reduced
slows disease progression