Pathology: Vasculidities

Question 1

What arteries does Giant Cell Arteritis generally affect, and what are the side effects of inflammation of each?

Answer 1

Branches of the Carotid:

1. Opthalmic - blindness/diplopia
2. Temporal - severe headaches, pain on temporal palpation
3. Facial - Jaw pain

Question 2

How do you treat Giant Cell Arteritis?

Answer 2

Corticosteroids or Anti-TNF therapies (robbins)

Question 3

You biopsy the temporal artery and find no abnormalities. Can you rule out giant cell arteritis?

Answer 3

NO. Giant cell arteritis is “notoriously patchy” along the length of the arteries.

Question 4

Describe what you’d have found histologically if your biopsy had been positive for giant cell arteritis.

Answer 4

Granulomatous inflammation within the inner media CENTERED ON THE INTERNAL ELASTIC MEMBRANE.

The giant cells have eaten some of it…

There are lymphocytes present as well (Th1’s that activate the MACs)

Question 5

Why is it important to rule out infectious etiology of vasculidities before treating?

Answer 5

The treatment for infectious vasculitis (rare) and immune-mediated is different!

You treat immune-mediated vasculitis with immunosuppressants like corticosteroids and cyclophosphamide.

Give these to a patient with an infectious vasculitis, and you’ll AID the disease process rather than treat it! Exacerbate the infection!

Question 6

Anti-proteinase 3 antibodies (c-ANCAs) are associated with what kind of vasculitis?

Answer 6

Wegener’s Granulomatosis

Question 7

Anti- Myeloperoxidase antibodies (p-ANCAs) are associated with which 2 vasculidities?

Answer 7

Microscopic Polyangiitis

Churg-Strauss Syndrome

Question 8

What’s the significance of ANCA presence in vasculitis?

Answer 8

ANCAs directly activate neutrophils, stimulating the release of reactive oxygen species and proteolytic enzymes, which may lead to endothelial cell injury.

Question 9

What’s the typical person who gets Takayatsu Arteritis?

Answer 9

Asian woman under the age of 50.

Presents with waning pulses, neurologic deficits, ocular disturbance, etc…

Question 10

What’s the other name for Takayatsu Arteritis? Why?

Answer 10

Pulseless disease

Takayatsu Arteritis is a granulomatous vasculitis of the aorta and its branches, causing transmural scarring and stenosis of the major vessels. This leads to markedly weak pulses in the upper extremities and the carotids.

Question 11

Are there ANCAs in Polyarteritis Nodosa?

Answer 11

NO Antibodies associated with it–> just Hepatitis B antigens.

Question 12

What is the ONLY organ that Polyarteritis Nordosa can’t touch?

What are the symptoms of the other ones getting attacked?

Answer 12

the Lungs.

Typically manifests in the kidneys, heart, liver, and GI tract vessels. –> side effects are hematuria, bloody stool, etc…

Skin also has nodules

Question 13

What does a histological examination of Polyarteritis Nodosa show?

Answer 13

1. PARTIAL involvement of the vessel lumen. Doesn’t affect the entire circumference.
2. Fibrinoid Necrosis

Question 14

A 3 year old child comes to you with fever, redness of his palms and soles, cervical lymph node enlargement, and symptoms of an MI. What does he have?

Answer 14

Kawasaki Disease

it looks like a virus! It’s not.

Question 15

What vessels does Kawasaki disease favor?

Answer 15

The coronary arteries. That’s why you see MI symptoms in a 4 year old.

Question 16

How do you treat Kawasaki disease?

Answer 16

IVIG and Aspirin

Question 17

What will you most likely die of if you don’t treat Polyarteritis Nodosa?

What percentage of people survive if you catch it and treat it?

Answer 17

renal failure - untreated

90% survival if treated

Question 18

What’s the other, more modern term for Wegener’s Granulomatosis?

Answer 18

Granulomatosis with Polyangiitis

Since Wegener was a pedo and all…. they took his name away

Question 19

WHere do you find giant cells in Wegener’s Granulomatosis?

Answer 19

In the PERIPHERY of the blood vessel, rather than the intima, like you find in Giant cell Arteritis.

Question 20

What 3 parts of the body does Wegener’s Granulomatosis affect?

Answer 20

Nasopharynx
Lung
Kidney

Question 21

What antibodies are found in Wegener’s Granulomatosis?

Answer 21

c-ANCAs

Question 22

How do you treat Weg’s Granulomatosis?

Answer 22

Cyclophosphamide

Question 23

What causes Kawasaki disease?

Answer 23

SUPERANTIGENS –> babies get a common infection that causes a non-specific immune reaction with tons of lymphocytes.

That’s why you give them IVIG. Aspirin covers the clot potential with vasculitis.

Question 24

What is an ectasia?

Answer 24

A birthmark. Technically it’s a dilation of a tubular structure besides blood vessels that are discrete and localized.

Question 25

What’s the difference (pathologically) between an aneurysm and an ectasia?

Answer 25

Ectasias are benign and clear up on their own. They DO NOT CREATE AREAS OF TURBULENT BLOOD FLOW.

Aneurysms do.