Vascular Pathology Pathoma Only Flashcards
Vasculitis is defined how?
Inflammation of a vessel wall
Wall of the blood vessel is composed of what layers?
Adventitia
Media
Intima
Intima is composed of what?
Endothelial cells sitting on a basement membrane
Adventitia is composed of what?
Connective Tissue
Media is composed of what?
Smooth muscle cells
How involved is infection with most vasculitis cases?
Not involved usually
What types of clinical findings do we see with vasculitis? 2 types
Nonspecific Symptoms
Symptoms of Organ Ischemia
Nonspecific symptoms of vasculitis are involved with what?
Fever, malaise, inflammation, weight loss, etc.
Two ways of getting organ ischemia due to vasculitis:
- Thrombosis - Endothelial cell damaged, causing coagulation cascade from subendothelial collagen and tissue factor, forming a thrombus that blocks up the vessel
- Inflame the wall, healing ensues, you get fibrosis of the wall, thus narrowing the lumen and decreasing blood flow to the organ, causing ischemic changes
Temporal (Giant Cell) Arteritis (TGCA) is most common in who?
Older adults, over the age of 50, females preferentially
Location of TGCAs?
Branches of the Carotid Artery
TGCA that causes headaches?
Temporal artery affected
TGCA that causes visual disturbances?
Opthalmic artery affected
TGCA that causes jaw claudication?
Arteries feeding muscles of the jaw
What other general symptoms doe we see with TGCA?
Flue like symptoms with joint and muscle pain
Lab findings for TGCA
Elevated ESR > 100
TGCA often involves getting a biopsy. What does that biopsy show?
- Inflammed vessel wall
- Giant cells present due to the fact that this is a granulomatous vasculitis
- Intimal fibrosis
TGCA is unique because it involves a long piece of vessel for biopsy. Why?
It’s segmental, not across the whole vessel. Thus a negative finding does not mean you don’t have it AND you have to check the whole thing because it could just be one small segment that is affected
Histological findings of TGCA
- Presence of Giant Cells
- Fibrosis and enlargement of the Intima
- Inflammation
How do we know we are looking at a Giant Cell?
Cell with many nuclei
What is the treatment for TGCA and what happens if we don’t treat it?
High risk of blindness without treatment due to the effects on the opthalmic artery, treat with corticosteroids
What is Takayasu Arteritis and how does it relate to TGCA? Who does it affect?
It’s another Granulomatous Arteritis but it affects adults <50 and is an asian female. Basically the same thing
There are a couple of differences between Takayasu and TGCA. What are they?
- Takayasu is more proximal and involves the aortic arch and its early branches
- Causes more general neurological and visual disturbances
What is Takayasu also called? Why?
Pulseless disease - Weak or absent pulse in the upper extremity
How do treatment and lab tests differ between TGCA and Takayasu?
They don’t. ESR still elevated and you still treat with corticosteroids.
Medium vessel vasculitis affects what types of arteries?
Muscular arteries specifically
Polyarteritis Nodosa (PN) involves what organs? What type of vasculitis is it?
Necrotizing vasculitis involving most organs but the lungs are spared.
Who gets PN?
Young Adults
Symptoms of PN? What arteries are affected in these symptoms?
All kinds of things, but usually:
- HTN (renal artery)
- Abdominal pain with melena (mesenteric artery)
- Neurologic disturbances
- Skin Lesions
PN is associated with serum ______.
HBsAg (Hep B surface Antigen)
There is something unique about PN tht we see on Biopsy. What is it?
“String of pearls”
The disease has various stages and they all appear segmentally along the wall.
What are the stages of Fibrinoid Necrosis (FN)?
Stage 1 - Fibrinoid Necrosis through all layers of the vessel (Transmural - through all layers)
Stage 2 - Fibrosis (healed Fibrinoid Necrosis!) - Vessel feels bumpy (gives name Nodosa)
Where does “String of pearls” come from
Different stages of FN through the vessel. In stage 1, we can get an aneurysm, a ballooning out, due to weakening of the wall from fibrinoid necrosis. This gives the “pearls”
Fibrinoid necrosis looks like what under histology?
Lots of pink! Inflammation also present
Treatment of PN? Penalties of not treating?
Corticosteroids AND Cyclophosphamide
Fatal if not treated
Kawasaki Disease affects who?
Asian kids <4
Presentation of Kawasaki?
Vague, Nonspecific symptoms
- Fever
- Conjunctivitis
- Red rash on palms and soles
- Enlarged cervical lymph nodes
Artery usually involved with Kawasaki? What bad complications (2 of them) are associated with it?
Coronary artery involvement most common.
- Thrombosis with myocardial infarction
- Aneurysm with rupture
Treatment of Kawasaki
Given Aspirin and IVIG
Aspirin is interesting because you don’t give kids Aspirin for viral infections, which this looks a lot like, due to the possibility of Reye’s Syndroms
Why do we give Aspirin for Kawasaki?
Stop platelet aggregation and thus stop Thrombus. Also inhibits TXA2 creation by the platelets.
What is Buerger’s disease
Necrotizing vasculitis of the digits (muscular arteries)
How does Buerger’s present?
Ulceration, gangrene, and autoamputation of fingers and toes