Trigger 6- IPF summary Flashcards
which people are the most prone to IPF
individuals with a mutation in SFTPC
which mutated gene can be a risk factor for IPF
SFTP
SFTP is a gene which encode
surfactant protein-c
without surfactant protein c
unfolded protein response by ER
alveoli are known as
pneumocytes
type 1 pneumocytes structure
squamous
- continous barrier between air and connective tissue
type 2 pneumocytes are
studded through type 1 cells
-cube shaped with microvilli
type 2 secrete
surfactant
surfactant
prevents the alveoli from collapsing during exhalation
T2 can divided into…
more T2 pneumocytes
or into T1 pneumocytes
between T1 and T2 pneumocytes and capillaries is
interstitial tissue
which cells are found in interstitial tissue
macrophages and fibroblast
outline pathology of IPF
1) when the alveolar lining is damage the T1 cells release TGF-B1
2) TGF-B1 stimulates the T2 cells to stimulates fibroblasts to differentiate into myofibroblasts
3) myofibroblast secrete reticular fibres and elastic fibres
4) myofibroblasts then apoptose
5) T2 cells start to over proliferate
6) leading to the stimulation of many fibroblasts
7) myofibroblasts stop apoptosing ad continue to make more and more collagen
8) collagen thickens the intersistial layer between the alveoli and capillaries
9) reducing the transfer of O2 into he lungs and Co2 out.
10) also causes the lugs to stiffen- harder for air to get in and out
IPF is characterised as a
restrictive interstitial lung disease
what do interstitial lung diseases decrease
- total lung capacity
- forced lung capacity
- FEV1
