Vasculitis

Question 1

What is vasculitis?

Answer 1

Heterogeneous group of disorders that all have inflammation of the blood vessel walls in common

Question 2

What is the problem with vascular damage?

Answer 2

It causes end organ damage

Question 3

How do the vasculitides present?

Answer 3

With fever/myalgias/arthralgias/malaise, then organ specific signs and symptoms

Question 4

Infectious vasculitis

Answer 4

Direct invasion of vessel wall by pathogens that result in inflammation.

Question 5

Fungal causes of vasculitis

Answer 5

Aspergillus, mucor

Question 6

Bacterial causes of vasculitis

Answer 6

Treponema pallidum, legionella, pseudomonas.

Rickettsia rickettsiae

Question 7

Non-infectious vasculitis

Answer 7

Not known to be caused by direct invasion of the vessel wall, perhaps immune mediated. q

Question 8

Why is it important to distinguish infectious/noninfectious vasculitis?

Answer 8

Because for noninfectious, immunosuppression could exacerbate infectious. Also know that noninfectous vasculitis can be precipitated by an infectious process.

Question 9

Important vasculitis classifications

Answer 9

Large medium small variable vessel vasculitis

Question 10

Medium vessels are…

Answer 10

Main visceral arteries, veins and their initial branches.

Question 11

Small vessels are..

Answer 11

intraparenchymal arteries, arterioles, capillaries, venules, and veins

Question 12

If a disease is classified as large vessel, or whatever, can it affect other vessels?

Answer 12

Yes.

Question 13

Some immunological mechanisms that can initiate non-infectious vasculitides

Answer 13

SLE: Dna-antiDNA complexes
Polyarteritis nodosa: HepBag and AB complexes
Streptokinase/penicillin are antigenic
Antiendothelial cell antibodies may predispose to kawasaki disease

Question 14

ANCA

Answer 14

Anti-neutrophil cytoplasmic antibody. Circulating that react with cytoplasmic antigens in neutrophils and endothelial cells. cytoplasmic anca is now Proteinase 3 anca (PR3anca). Perinuclear anca is now MPO-anca (myeloperoxidase).

Question 15

Blindness in giant cell arteritis due to

Answer 15

Infection of small vessels ,even though GCA is a large artery disease.

Question 16

Two major variants of LVV

Answer 16

Giant Cell Arteritis (>50)
Takayasu Arteritis (<50)

Histopathologically indistinguishable – granulomatous

Question 17

Granuloma characteristics

Answer 17

Has giant cells, epithelioid macrophages and a collar of lymphocytes

Question 18

How to diagnose GCA

Answer 18

Temporal artery biopsy.

Question 19

Does a negative biopsy rule out GCA?

Answer 19

No. Start steroids

Question 20

Biopsy appearance for GCA

Answer 20

Intimal thickening with granulomatous inflammation. IEL fragmentation with elastic stain.

Question 21

Where does takayasu arteritis present

Answer 21

Left subclavian, left common carotid, brachiocephalic artery

Question 22

How to diagnose?

Answer 22

Can’t do biopsy because of the vessels involved. Signs of limb ischemia, decreased pulse at brachial arteries, blood pressure difference between arms, subclavian bruits

Question 23

Patients with Takayasu?

Answer 23

80-90% females. Under 40.

Question 24

How to treat takayasu arteritis?

Answer 24

Glucocorticoids early, vascular surgery later.

Question 25

Medium vessel vasculitis onset compared to LVV

Answer 25

Onset in more acute and necrotizing than in LVV.

Question 26

Two major categories of MVV

Answer 26

Polyarteritis nodosa

Kawasaki’s disease

Question 27

Polyarteritis nodosa

Answer 27

Necrotizing vasculitis of medium to small arteries (ONLY!). Often leads to inflammatory aneurysms or rupture

Question 28

Most commonly involved arteries in polyarteritis nodosa?

Answer 28

Renal arteries. causing hypertension and ischemic nephropathy But not glomerular capillaries. So no glomerulonephritis

Question 29

What set of arteries does PAN usually spare?

Answer 29

Pulmonary circulation

Question 30

Symptoms of PAN

Answer 30

Malaise fever weightloss, hypertension, abdominal pain/melena, diffuse muscular aches and pains.

Question 31

How to treat PAN?

Answer 31

Corticosteroids and cyclophosphamide

Question 32

Is PAN assoiciated with ANCA?

Answer 32

NO! 30 % have hep B

Question 33

Biopsy findings in PAN

Answer 33

Begins as a segmental transmural inflammation with neutrophil predominance. Later, fibrinoid necrosis (bright pink) comprised of deposits of immune complexes.Lots of fibrin too. Finally chronic inflammation and scarring. All stages of activity present within the same vessel!

Question 34

Kawasaki disease

Answer 34

Medium size arteries. Is associated with mucocutaneous lymph node syndrome and is found in infants and young childnre. Acute febrile and self limited illness. But has predelection for coronoarry arteries.

Question 35

How does Kawaksaki disease happen?

Answer 35

Autoantibodies to endothelial and smooth muscle cells

Question 36

Signs and symptoms

Answer 36

Fever and mucocutaneous lymph node senydome. Conjuctnival injection oral mucositis with strawberry ongue and cracked red lips. Accompanied by a skin rask and edema of hands and feet. Cerbival lymph node enalrgement.

Question 37

How many people w/ KD develop cardiac sequellar?

Answer 37

20% if untreated. Can cause coronary artery ectasia leading to aneurysms and can cause rupture or thrombosis causing MI.

Question 38

How to treat KD

Answer 38

ASPIRIN!!! This is super high yield.

Question 39

Not doing small vessels because

Answer 39

I’m lazy!