Trigger 6: IPF management and treatment

Question 1

Management pathway for IPF patients

Answer 1

1) adult with IPF
2) assess pulmonary rehab programme tailored for IPF
3) Offer best supportive care
4) offer drugs to treat IPF and withdraw ineffective therapies
5) explore lung transplant
6) review and follow-up
7) end of life care

Question 2

successful management is dependent o n

Answer 2

establishing diangosis

Question 3

monitoring test

Answer 3

o	Forced vital capacity
o	Diffusion capacity of lung for CO (DLco)
o	FEV1/FVC ration
o	Oxyhaemoglobin sats
o	6-minute walk test

Question 4

thoracic imaging

Answer 4

HRCT to measure severity of fibrosis

Question 5

non-pharmaceutical treatment

Answer 5

• self care
• oxygen therapy
• pulmonary rehab
• lung transplant

Question 6

self care

Answer 6

• stop smoking

- pneumococcal and seasonal influenza vaccine

Question 7

oxygen therapy

Answer 7

o Nasal tube or mask and oxygen concentrator

o Raises oxygen saturation levels

Question 8

pulmonary rehab

Answer 8

• education
• PE and breathing exercises
• advice on nutrition
• social support network

Question 9

lung transplant

Answer 9

only certain candidates appropriate

Question 10

survival rate of lung transplant

Answer 10

50% year survival rate following transplant

Question 11

name 5 pharmaceutical treamtents

Answer 11

Nintedanib

Pirfenidone

Ganciclovir

N-acetyl-cysteine or NAC

Lebrikizumab

Question 12

Nintedanib is a

Answer 12

tyrosine kinase inhibitor

Question 13

which receptors does Nintedanib inhibit?

Answer 13

PDGFR, FGFR, VEGFR

Question 14

PDGFR

Answer 14

platelet-derived growth factor receptor

Question 15

FGFR

Answer 15

fibroblast growth factor receptor

Question 16

VEGFR

Answer 16

vascular endothelial growth factor

Question 17

main mechanism of action of Nintedanib- basic

Answer 17

nintedanib reduces disease progression of idiopathic pulmonary fibrosis and slows the decline in lung function by blocking the signalling pathways that are involved in fibrotic processes.

Question 18

Pirfenidone is an

Answer 18

antifibrotic agent

Question 19

Ganciclovir

Answer 19

anti-viral therapy

- Been demonstrate that a two week therapy may temporarily attenuate disease progression

Question 20

N-acetyl-cysteine or NAC

Answer 20

Breaks up mucus in the lungs (medication to help with symptoms)

Question 21

drugs in trial

Answer 21

Lebrikizumab

Question 22

Lebrikizumab

Answer 22

anti IL-13 - inflammation?

Question 23

NAC inhibits

Answer 23

oxidative stress (which leads to epithelial cell damage)

Question 24

Pirifidone inhibits

Answer 24

• oxidative stress
• Type 2 cell proliferation, cell migration and Th2 dominant response
• growth factor and protease activation, neovascularisation
• fibroblast recruitment, proliferation and myofribroblast differentiation

Question 25

Ninteedanib

Answer 25

• growth factor and protease activation, neovascularisation

- fibroblast recruitment, proliferation and myofribroblast differentiation

Question 26

outline IPF disease pathway

Answer 26

1) virus, acid reflux smoking etc causes injury to epithelial cells
2) injury and death to epithelial cells
3) Type 2 cell proliferation and cell migration, T2 dominant immune milieu
4) growth factor and protease activation, neovasculization
5) fibroblast recruitment, proliferation and myofibroblast differentiation
6) matrix accumulation and cross-linking