Respiratory Pathology 6 - SRS Flashcards
What are the categories of pulmonary diseases of vascular origin we covered in this lecture/dsa?
Pulmonary embolism
Pulmonary hemorrhage
Pulmonary Infarction
Pulmonary HTN
Diffuse Pulmonary hemorrhage syndromes
Other vasculitidies
What are the diffuse pulmonary hemorrhage syndromes we covered here?
- Goodpasture syndrome
- Wegener Granulomatosis
- Idiopathic pulmonary hemosiderosis
Pulmonary embolism is an underdiagnosed and significant cause of death via what?
Respiratory and hemodynamic compromise with 10% of cases resulting in infarction with hemoptysis.
What color infarct do you get in the lungs?
Red infarct, because various sources of blood to the parenchyma
What are the clinical presentations associated with pulmonary embolism?
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- Dyspnea
- Fever
- Chest pain
- hemoptysis
- electromechanical dissociation
- Sudden death
What enzyme will be elevated in PE?
LDH (as with many other diseases)
Where do most PE’s come from, and where do they most often end up?
Most form in the deep leg veins
Most go to the lower lobe of the lungs
What is the procedure by which we perform a V/Q scan?
- Patient inhales a radioactive compound
- Radioactive compound injected into the vein, travels to the lung tissues
- Visualize the distribution of the two radioactive compounds to see if areas are subject to obstruction of vasculature or airway
In a pulmonary embolus what would you likely see if you performed a V/Q scan?
- Inhaled radioactive compound will likely distribute evenly to the whole lung.
- Injected radioactive compound will reveal a portion of lung that is not being perfused due to clot obstruction
What is the gold standard for dxing a PE?
Pulmonary angiography
What is shown on this spiral CT?
Pulmonary embolisms indicated by both arrows. While angiography is the gold standard, typically in clinic the diagnosis is made with spiral CT
What lines will you see in a thrombus that tell you it did not form post mortem?
Line’s of Zahn
What is shown here?
Embolic blockages at the points indicated.
What is shown here?
An acute hemorrhagic pulmonary infarct
Explain the color of this infarct.
This is an older infarct that has had hemosiderophages come in and clean out some of the RBC’s. So, not a white infarct even though it looks white now.
What caused the finding indicated by the arrow?
Fat embolus d/t trauma (e.g. long bone fx or surgery)
The finding is absence of RBC’s in the vessel lumen. The staining procedure dissolved the fat embolus, so you can’t see it either. Have to logic it out.
This image has been frozen sectioned and stained with Sudan Black B. What is shown here?
This is a fat embolus. The process of frozen sectioning preserves the fat from the embolus, and a stain such as Sudan Black B will visualize the fat cells.
In addition to fat emboli, what other embolism is possible from a long bone fx??
Bone marrow embolus
Name as many types of emboli as you can!
- Thrombotic
- Fat
- Bone marrow
- Tumor
- Air
- Nitrogen
- Talc and metal oxides
- bullets
- amniotic fluid
What kind of embolism is shown here?
What are the lines?
Amniotic Fluid embolus, occured during delivery due to tear in the placenta allowing the amnion into the circulatory system.
The lines are Vernix - shed superficial epithelium.
This picture wiggled
What is a possible consequence of this?
This will lead to production of tissue factor and will often lead to concomitant disseminated intravascular coagulation.
Medical emergency
What does the chest xray of the patient this embolus was taken from tell us about the patient’s status?
The lung fields are diffusely edemetous and inflamed and the mother is in danger of going into acute respiratory distress syndrome d/t the amniotic embolus.
At what pressure is the pulmonary system in HTN?
Pressures are greater than or equal to 25mmHg
What are the clinical symptoms of PHTN?
- Dyspnea
- fatigue
- anginal chest pain
- cyanosis if advanced
What are two long term consequences of pulmonary hypertension?
Cor Pulmonale
Cardiac failure
What are the groups of pulmonary hypertension?
there are 5 now.
- Pulmonary arterial HTN
- PHTN due to left heart fail
- PHTN d/t lung parenchymal disease or hypoxemia
- D/t chronic thromboembolic state
- pulmonary hypertension of multifactorial basis
What are the causes Gomex lists for group one pulmonary arterial hypertension?
- Primary idiopathic pulmonary arterial HTN
- autoimmune connective tissue disorders
- drugs
- fenfluramin/phentermine
- bush tea
- aminorex
- toxic rapeseed oil
- dexfenfluramine
What conditions lead to group 2 phtn?
Cardiac malformations
acquired heart disease
What conditions lead to group 3 phtn?
Chronic obstructive or interstitial lung diseases
obstructive sleep apnea
What is the mechanism of the heritable primary pulmonary hypertension that we know about?
Mutation of the bone morphogenic protein receptor type 2 (BMPR2).
What does the BMPR2 do in vascular smooth muscle cells? What does its mutation lead to?
Inhibits proliferation and favors apoptosis.
Mutation inactivates, thus favoring SMC proliferation.
In secondary pulmonary HTN what are the results from the increased pressures?
Significantly increased presure from any cause leads to medial hypertrophy, which narrows the arteries such that pressure is further elevated.
What are the vascular changes seen in pulmonary hypertension?
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- Atheroma formation in the large vessels
- marked medial hypertrophy
- plexiform lesion of small arteries characteristic of advanced pulmonary HTN
What are some causes of diffuse alveolar hemorrhage syndrome?
- Goodpasture syndrome
- idiopathic pulmonary hemosiderosis
- Vasculitis
- Wegeners
- hypersensitivity pneumonitis
- lupus erythematosis
Describe the pneumonitis that occurs in goodpasture syndrome.
Necrotizin hemorrhagic interstitial pneumonitis
Goodpasture syndrome is causes a necrotizing hemorrhagic interstitial pneumonitis and presents with renal involvement. What is the progression of the renal involvement?
What are the auto-antibodies against?
- Focal proliferative rapidly progressive to crescentic glomerulonephritis
- Anti-basement membrane antibodies to the α3 chain of collagen IV.
What is the most common cause of death in Goodpasture’s syndrome?
What is the treatment?
Renal failure (uremia)
Plasmapheresis and immunosupression to treat
In what populations is goodpastures most likely?
On exam questions what is the tip off presentation in the stem?
•M>F, young smokers (teens-20s)
Presence of pulmonary SOB, coughing up blood, renal failure with elevated creatinine.
What is shown here?
Goodpastures, linear deposition of anti-basement membrane antibodies (ABMA)
The patient this image is from has a rare condition that usually affects children and causes episodes of diffuse hemorrhage.
Based on the findings shown attached, with the prussian blue stain at right, what disease does this person have?
Idiopathic pulmonary hemosiderosis
Note the diffuse hemosiderin deposition in the macrophages and alveolar walls.
What antivascular antibodies are associated with idiopathic pulmonary hemosiderosis?
What are four common symptoms associated with this illness?
What is the treatment?
• No antivascular antibodies have been found
- Productive cough
- hemoptysis
- anemia
- weight loss
Immunosuppression works as the treatment, for totally unknown reasons!
What is the classic triad for polyangiitis with granulomatosis?
Sinusitis
Lung involvement
Renal involvement
What areas undergo necrosis in Wegener’s?
Necrotizing granulomatous arteritis of the lung
necrotizing inflammation of the Upper respiratory tract
necrotizing (crescentic) glomerulonephritis
What is the diagnostic technique for Wegners?
Treatment?
•Cytoplasmic pattern anti-neutrophil antibodies (PR3/c-ANCA) in 85%
Immuno Rx (variable prognosis)
