6 - lung fibrosis treatment Flashcards
methods to monitor IPF progression
pulmonary function tests
thoracic imaging
example of types of pulmonary function tests
forced vital capacity diffusion capacity of the lungs forced expiratory volume (FEV) oxyhaemoglobin saturation 6-minute walk test
method for thoracic imaging
high resolution CT scan
only possible cure for IPF
lung transplant
why is palliative care important for IPF
IPF is almost always a terminal disease
methods to diagnose IPF
listening for crackles through stethoscope
thoracic imaging
lung function tests
BAL fluid
endoscopic lung biopsy
results of 6 min walk test
often only 250m for IPF patients
should be around 700m
treatment strategies for IPF
proton pump inhibitor (PPI)
anti-coagulants
pharmaceuticals
pulmonary rehabilitation
example of proton pump inhibitor (PPI) to treat IPF
omeprazole
aim of omeprazole
proton pump inhibitor
reduce stomach acid made in the stomach for reflux to reduce coughing
types of pharmaceutical therapies used to treat IPF
tyrosine kinase inhibitor (nintedanib)
antifibrotic agent (pirfendione)
antiviral therapies (ganciclovir)
nintedanib
RTK inhibitor
blocks downstream signalling by inhibitiing cytokine receptors
slows disease progression
mechanism underlying nintedanib
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
testing for the presence of viruses in IPF
molecular techniques such as PCR, qPCR
testing for presence of antibodies –> shows past exposure to virus
why is therapy for IPF important
no realistic cure currently
main aim is to relieve symptoms and slow progression
pirfenidone
slows development of scarring in the lungs by reducing the immune response
N-acetylcysteine
slows progression of IPF
anti-oxidant
loosens thick mucous
other potenetial targets for anti-fibrotic drugs
CYTOKINES (IL-3, IL-21), chemokines angiogenic factors (VEGF) growth fators acute phase proteins caspases
non-pharmaceutical treatment methods
improve lifestyle
oxygen therapy
pulmonary rehabilitations
lung transplant
methods to improve lifestyle
–> stop smoking, exercise regularly, eat healthily, have seasonal vaccinations
pulmonary rehabilitation methods
physical exercises
breathing exercises e.g.singing
psychological and social support
education about IPF
importance of oxygen therapy
blood oxygen decreases in IPF causing breathlessness
treatment allows patient to be more active
method of oxygen therapy
via nasal tube or mask attached to machine
current clinical trials for IPF treatment
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
ganciclover
anti-viral therapy
shows promising effects when combined with standard therapy
why are animal studies important
used to demonstrate how, during latency, herpesvirus infections reprogram lung epithelial cells to produce profibrotic factors making lungs more susceptible to fibrotic insult
median survival of IPF patients
2-3 years
common name for PPI drugs
antacids
FGF
fibroblast growth factor
effect of nintedinab action
fibroblast proliferation is inhibited and inflammation is reduced
slows disease progression