Vasculitis

Question 1

What are the immune complex mediated small vesselvasculitis diseases?

Answer 1

hypersensitivity vasculitis;
Henoch-Schonlein purpura;
cryoglobulinemic vasculitis

Question 2

Define vasculitis

Answer 2

inflammation of blood vessels with necrosis of blood vessels walls, narrowing, and occlusion

Question 3

How to classify vasculitis?

Answer 3

size of blood vessels involved, pattern of organ involvement, pathophysiology

Question 4

Give the size of the blood vessel involvement

Answer 4

small=> capillaries/postcapillary venules
Medium=>muscular arteries and arterioles
large=>aorta and branches

Question 5

What vasculitis disorders affect the aorta?

Answer 5

Giant cel arteritis

Takayasu’s arteritis

Question 6

What vasculitis disorders affect large to medium sized arteries

Answer 6

Polyarteritis nodosa

Kawasaki disease

Question 7

What vasculitis disorders affects the arteriole/capillary junction?

Answer 7

anti-GBM (Goodpasture’s disease)

Question 8

What vasculitis disorders affect the capillary and venule?

Answer 8

leukocytoclastic vasculitis

Question 9

What vasculitis disorders affect arterioles and venules?

Answer 9

Henoch Schonlein purpura;

cryoglobulinemic vasculitis

Question 10

What vasculitis disorders affect small sized arteries to venules?

Answer 10

microscopic polyangitis

Question 11

What vasculitis disorders affect small sized arteries to veins?

Answer 11

granulomatosis w/ polyangitis;

Churg-strauss syndrome

Question 12

What connective tissue disorders are associated with IC-mediated vasculitis?

Answer 12

SLE;
Sjogren’s syndrome’
RA

Question 13

What is the classic cutaneous finding in small vessel vasculitis?

Answer 13

palpable purpura

Question 14

What typically causes hypersensitivity vasculitis?

Answer 14

a reaction to medication or infection

Question 15

What are the clinical manifestations associated with hypersensitivity vasculitis?

Answer 15

purpura, arthritis, glomerulonephritis, colicky abdominal pain

IgA deposition w/in blood vessel walls

Question 16

Differentiate cryoglobulinemic vasculitis and mixed cryoglobulinemia

Answer 16

CV=> long standing HCV infection

MCV=> immuno reactants involved include IgG and IgM

Question 17

Define cryoglobulins

Answer 17

Abs that precipitate from serum under conditions of cold and resolubilize upon warming

Question 18

What is characteristic of type I cryoglobulins

Answer 18

monoclonal, RF negative and associated w/ malignancies

cause hyperviscosity syndromes => NOT vasculitis

Question 19

What is characteristic of type II and IIIcryoglobulins

Answer 19

polyclonal (IgG and IgM Abs) have RF activity;

RESULT IN IC-MEDIATED VASCULITIS

Question 20

Which cryoglobulins are associated with hep C?

Answer 20

type II and III

Question 21

Cutaneous vasculitis in connective tissue disorders are associated with what?

Answer 21

hypocomplementemia;
high titers of ANAs;
Biopsy shows IgG and C3 deposition

Question 22

Why must rheumatoid vasculitis (RV) and periungual vasculitis be differentiated?

Answer 22

vasculitis-specific therapy

Question 23

What sized vessels does RV associated with?

Answer 23

aggressively involves medium and small vessels

Question 24

What is the primary difference in polyarteritis nodosa and RV?

Answer 24

microaneurysms are more common in PAN

Question 25

RV is classically found in what patients?

Answer 25

RA patients with nodular, RF positive, joint destructive disease w/ few clinical indications of active synovitis at onset

Question 26

Most common presentation in RV?

Answer 26

pupuric lesions w/ or w/o concomitant medium vessel vasculitis, deep cutaneous ulcer over malleoli are hallmarks of RV

Question 27

Characterize reversible cerebral vasoconstrictive syndrome

Answer 27

acute onset headaches and reversible cerebrovasoconstriction w/ multiple areas of smooth or tapered arterial narrowinf followed by segments of normal-caliber arteries

leading mimic of PACNS

Question 28

What is Behcets disease?

Answer 28

complex multisystem disease characterized by oral and genital ulcers

Question 29

In Behcet’s disease, how could you prove it is cutaneous?

Answer 29

cutaneous lesions should display a neutrophilic vascular rxn on histo exam