4 Flashcards
(37 cards)
connective tissue disease-ILD :
what are some of the most common connective tissue diseases that come with this
systemic sclerosis, rheumatoid arthritis and idiopathic inflammatory myositis
what is IPAF (interstitial pneumonia with autoimmune features)
ILD can also occur in the presence of autoimmune features that do not meet classification criteria for a specific CTD.
CTD-ILD radiology:
what areas of the lung will this affect
what can be seen on the scan
which CTD is the UIP pattern most common in and what does having UIP mean
what pattern do most CTD patients have
affects central areas of the lung
honeycombing at the periphery
the UIP pattern is most common in rheumatoid arthritis.
most CTD patients have NSIP pattern
if you have UIP it means you are less likely to survive because it means fibrosis
NSIP vs UIP
NSIP means inflammation and can be treated
UIP means fibrosis and is more severe
what is the pathogenesis of CTD-ILD
it is not known.
but one theory is that an unknown trigger causes inflammatory cells to invade the interstitial and alveolar spaces and causes epithelial damage.
This will recruit fibroblasts and myofibroblasts which make ECM proteins and lead to fibrosis.
what is sarcoidosis and what organs are affected
a granulomatous disease characterised by the presence of non coasting granulomas involving multiple organ systems.
lungs are affected in 90% of cases
what is the pathogenesis of sarcoidosis and what happens when the antigen is detected
how does this cause fibrosis
the antigen is detected and internalised and taken to the lymph nodes.
CD4 cells are activated and Th1 is induced by the antigen.
This will activate macrophages to organise into a granuloma and chronic inflammation is an important risk factor for fibrosis.
what are the irritants causing sarcoidosis
what does the radiology look like
insecticides, farmers, mould, jobs with musty odours.
radiology shows enlarged lymph nodes and nodules form which appear white.
where is the CFTR protein found and what does it transport
what does the mutation cause
on all membranes that make mucus (lungs, bowels)
it transports Cl- out of the cell
mutations cause thicker mucus
what are the lungs like when a CF patient is born and how does this change throughout life
they have structurally normal lungs but due to the thick mucus the airways are inflamed and they have bowel congestion problems.
This will eventually lead to bronchiectasis and then fibrosis and airway remodelling.
how can CF be diagnosed
check genetic profile
raised skin salt
heal prick test
can be diagnosed late when they go to infertility clinics
what complications come with CF
what problems come with taking lots of antibiotics
bronchiectasis infertility malabsorption CF related diabetes liver disease
tolerance, allergies, renal impairment.
what are some treatments for CF
segregated to stop infections anti inflammatories (azithromycin and steroids) airway clearance physiotherapy exercise nutritional support lung transplant vaccinations
what are advantages of personalised medicine
people take it more often if its working
improve quality of life
minimise drug reactions
lowers healthcare cost
what are the classes of CFTR defect
1- no CFTR is made 2- CFTR can't be trafficked 3- CFTR reached surface but can't open 4- decreased conductance 5- reduced synthesis
what is ivakaftor and what does it do
CFTR potentiator so it helps CFTR open when it gets to the cell surface
so it helps the class 3 mutations
but It is very expensive
what is orkambi made up of and what does it do
it is approved
what is a similar drug that has less side effects
what mutation needs this
ivakaftor and lumacaftor
lumakaftor is a CFTR corrector and helps trafficking problems it will help the class 2 mutations
its not approved because its too expensive and has side effects, so it is only given on compassionate use which is when you have a 40% decline in lung function
symkevi
508 90% of people
what is triple therapy used for and what does it contain
is it approved
90% of CF patients
contains symkevi and VX445
not approved yet and very expensive
what are some of the challenges in finding a treatment for CF
adherence to treatment high treatment burden high cost allergies to treatments side effects Psychological issues
what did the PANTHER study find
you are more likely to die from combination therapy (steroids and immunosuppressant) than doing nothing if you have IPF
what does pirfenidone do
what are the side effects
what did the CAPACITY and ASCEND study find
stops proliferation of fibroblasts and TGFB and the synthesis of ECM proteins
can cause photosensitivity (skin rash), gastrointestinal upset, and liver abnormalities.
found pirfenidone caused a reduced decline in FVC
what has nintendinab already been licensed for
side effect?
how does it work
what did the IMPULSIS study find
cancer
diarrhoea, liver abnormalities
inhibitor of multiple tyrosine kinase receptors
reduced decline in FVC
what can pirfenidone and nintendinab be uses to treat
ILD (mainly IPF)
what is the criteria for ILD progression
they had to have a decline in FVC of 10% or more
or an FVC decline of 5-10% with worsening symptoms
or increased fibrosis on HRCT
