Idiopathic pulmonary fibrosis (IPF) Flashcards
There are 2 types of restrictive lung disease. Which 2 of the following are the correct categories of restrictive lung diseases?
1 - interstitial
2 - alveolar fibrosis
3 - extra pulmonary
4 - intra pulmonary
1 - interstitial
- lung tissue is affected and loses elastic properties
3 - extra pulmonary
- affects tissue outside the lungs, such as obesity and scoliosis
In a restrictive lung disease, do patients have difficulty in inhalation, exhalation or both?
- inhalation
In restrictive lung diseases the elastic tissue of the lungs is affected. Are both recoil and compliance of lung tissue reduced in asthma?
- no
- lost of elastic properties, so lungs cannot expand properly
Looking at the alveolar in the image, label the image using the labels below:
- interstitial space
- type 1 pneumocytes
- type 2 pneumocytes
- pulmonary capillaries
- surfactant
- macrophages
- fibroblasts
1 - type 1 pneumocytes
2 - pulmonary capillaries
3 - macrophages
4 - fibroblasts
5 - interstitial space
6 - surfactant
7 - type 2 pneumocytes
In spirometry, would we expect to see an increase of decrease in functional residual capacity (FRC) (remaining air in lungs at end of normal exhalation) in a patient with a restrictive lung disease?
- decreased as lungs are typically smaller
- recoil = increased (ability of lungs to snap back and exhale air)
- compliance = decreased (lungs unable to stretch)
In spirometry, would we expect to see an increase of decrease in forced vital capacity (FVC) (air that can forcefully expired following maximum inhalation) in a patient with a restrictive lung disease?
- significant reduction
- compliance is reduced so patients lungs do not expand properly
In spirometry, would we expect to see an increase of decrease in forced expiratory volume in 1 second (FEV1) (air that can forcefully expired in 1 second following maximum inhalation) in a patient with a restrictive lung disease?
- mildly reduced
What is the ratio that is diagnostic in patients with a restrictive lung disease?
1 - FVC/FEV1 >90%
2 - FVC/FEV1 >80%
3 - FVC/FEV1 >75%
4 - FVC/FEV1 >60%
3 - FVC/FEV1 >75%
- both FVC and FEV1 are reduced but FVC is reduced more
In patients with a restrictive lung disease is the total lung capacity increased or decreased?
- decreased
- lungs can become smaller
In a healthy alveoli, when the alveolar is damaged by a stressor or infection, the type 1 pneuomcytes secrete what?
1 - IL-6
2 - TNF-a
3 - TGF-B1
4 - TGF
3 - TGF-B1
- tissue transforming growth factor B1
In a healthy alveoli, when the alveolar is damaged by a stressor or infection, the type 1 pneuomcytes secrete tissue transforming growth factor B1. This in turn stimulates the type 2 pneumocytes to do what?
1 - increase the secretion of surfactant
2 - increase mucus production
3 - stimulate fibroblasts to proliferate
4 - stimulate macrophages to proliferate
3 - stimulate fibroblasts to proliferate
- fibroblasts become myofibroblasts
- myofibroblasts secrete reticular fibres
and elastic fibres
- myofibroblasts undergo apoptosis when stressor/infection is removed
Is idiopathic pulmonary fibrosis classed as an idiopathic interstitial pneumonia (IIP) or a Diffuse Parenchymal Lung Disease (DPLD)?
- IIP
- as we do not know the exact cause
What is the most common cause of idiopathic interstitial pneumonia (IIP)?
1 - Non-specific interstitial pneumonia
2 - Idiopathic pulmonary fibrosis
3 - Pulmonary Alveolar Proteinosis
4 - Acute interstitial pneumonia
2 - Idiopathic pulmonary fibrosis
In idiopathic pulmonary fibrosis, also called restrictive lung disease, the type 2 pneuomcytes over proliferate. This in turn stimulates fibroblasts to secretes excessive reticular and elastic fibres. Furthermore the myofibroblasts do not undergo apoptosis. Does this increase or decrease the interstitial space thickness?
- thickens
- results in the lungs becoming stiff and the patient becoming breathless
In idiopathic pulmonary fibrosis, also called restrictive lung disease, there is a thickening of the interstitial space. What affect does this have on ventilation (V) / perfusion (Q) ratios?
- causes V/Q mismatch
- air can come in but cannot diffuse efficiently across thickened interstitial space
In idiopathic pulmonary fibrosis (IPF), also called restrictive lung disease, there is a thickening of the interstitial space. causing a ventilation (V) / perfusion (Q) mismatch. Why do we generally not see type 2 respiratory failure in IPF?
1 - O2 is able to diffuse more effectively
2 - CO2 is able to diffuse more effectively
3 - O2 binds with fibroblasts
4 - O2 cannot leave the capillaires
2 - CO2 is able to diffuse more effectively
- O2 cannot diffuse properly
- in severe cases it can cause type 2 respiratory failure
What might we be able to see on an an X-ray in patients with idiopathic pulmonary fibrosis?
1 - bilateral haziness
2 - small lungs
3 - blunted costophrenic angles
4 - reticular nodules at base of lungs
5 - all of the above
4 - all of the above
- reticulo = refers to causing a net like appearance
- nodular = refers to the appearance of nodules, many small round opacities
On a Ct scan, what may be present in patients with idiopathic pulmonary fibrosis?
1 - ground glass changes
2 - parenchymal changes
3 - hilar lymphadenopathy
4 - honeycombing and traction bronchial dilation
4 - honeycombing and traction bronchial dilation
- bilateral
- peripheral
- basal
- multi layered
