Lung Fibrosis Flashcards
what are the 5 type of cells present in the bronchial epithelium?
- ciliated
- goblet
- basal
- intermediate
- Clara
what are the two types of cell present in alveolus?
- Type I cell
2. Type II cell
what is the mechanism of breathing in/inspiration?
Inspiration
- External Intercostals - contract
- Internal Intercostals - relax
- Ribs and Sternum move upwards and outwards
- Diaphragm contracts and lowersIncrease in chest depth & volume
- As Lungs expand the pressure inside the lungs decreases
- Therefore air rushes in, to balance pressure difference - External intercostal contract and ribs lift up and out
- Diaphragm flattens + increases thoracic volume
what is the mechanism of expiration/breathing out?
Expiration
- relaxing the inspiratory muscles
- Gravity lowers ribs + sternum
- Lungs recall as diaphragm relaxes + returns to dome shape
- Reduced space in thorax increases air pressure
- Air in lungs is forced out
- Intercostals relax + ribs return to resting position
- Diaphragm re-domes + decreases thoracic volume
what happens to the following in breathing in and out?
- chest
- diaphragm
breathing in 1. chest - expands 2. diaphragm- contracts breathing out 1. chest - contracts 2. diaphragm - relaxes
what is pulmonary fibrosis?
it is the end stage of a heterogenous group of interstitial lung disease
what is survival rate for IPF?
mean survival is 2-3 years
what is fibrosis?
accumulation of extracellular matrix
how is an IPF lung different from normal lung?
- scarred tissue- pale looking
- healthy tissues replaced by excessive matrix
- distorted airways - vulnerable to infection
what are the symptoms of IPF?
- dyspnoea - shortness of breath
- coughing (dry)
- fever
- weight loss
- clubbing (thickening of finger tips)
what does the physical examination consists of in IPF?
lung sounds -
- velcro sounds
- pleural rub
what are the management techniques for a patient with IPF?
Key management decisions
- administration of pharmacological agents
- monitoring of the disease (how)?
- decision of whether a patient should be refried for lung transplant
- state of the disease for e.g. whether it is the end stage and unlikely to respond to therapies then providing plaintive care is best approach
how is IPF monitored?
- pulmonary function tests
2. thoracic imaging - HRCT to measure the severity of disease
what does pulmonary function test include?
- FVC
- oxyhemoglobin saturation
- 6-minute test
- DLCO - lung diffusion capacity for CO
- FEV1/FVC ratio
briefly outline the pathomechanism for IPF
- epithelial cell injury |
- inflammation coagulation cascade activation (TF/FVIIa/FXa-> thrombin)
- establishment of chemokine networks, leukocyte infiltration and AEC proliferation (chemokine, ROS, TGFbeta, PDGF)
- fibroblast recruitment, proliferation and differentiation
- loss of organ function