Trigger 6: IPF management and treatment Flashcards
Management pathway for IPF patients
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
successful management is dependent o n
establishing diangosis
monitoring test
o Forced vital capacity o Diffusion capacity of lung for CO (DLco) o FEV1/FVC ration o Oxyhaemoglobin sats o 6-minute walk test
thoracic imaging
HRCT to measure severity of fibrosis
non-pharmaceutical treatment
- self care
- oxygen therapy
- pulmonary rehab
- lung transplant
self care
- stop smoking
- pneumococcal and seasonal influenza vaccine
oxygen therapy
o Nasal tube or mask and oxygen concentrator
o Raises oxygen saturation levels
pulmonary rehab
- education
- PE and breathing exercises
- advice on nutrition
- social support network
lung transplant
only certain candidates appropriate
survival rate of lung transplant
50% year survival rate following transplant
name 5 pharmaceutical treamtents
Nintedanib
Pirfenidone
Ganciclovir
N-acetyl-cysteine or NAC
Lebrikizumab
Nintedanib is a
tyrosine kinase inhibitor
which receptors does Nintedanib inhibit?
PDGFR, FGFR, VEGFR
PDGFR
platelet-derived growth factor receptor
FGFR
fibroblast growth factor receptor
VEGFR
vascular endothelial growth factor
main mechanism of action of Nintedanib- basic
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.
Pirfenidone is an
antifibrotic agent
Ganciclovir
anti-viral therapy
- Been demonstrate that a two week therapy may temporarily attenuate disease progression
N-acetyl-cysteine or NAC
Breaks up mucus in the lungs (medication to help with symptoms)
drugs in trial
Lebrikizumab
Lebrikizumab
anti IL-13 - inflammation?
NAC inhibits
oxidative stress (which leads to epithelial cell damage)
Pirifidone inhibits
- oxidative stress
- Type 2 cell proliferation, cell migration and Th2 dominant response
- growth factor and protease activation, neovascularisation
- fibroblast recruitment, proliferation and myofribroblast differentiation
Ninteedanib
- growth factor and protease activation, neovascularisation
- fibroblast recruitment, proliferation and myofribroblast differentiation
outline IPF disease pathway
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
