vasculitis

Question 1

what is vasculitis

Answer 1

inflammation of blood vessels; arteries, arterioles, veins, venules or capillaries

Question 2

what can vasculitis lead to

Answer 2

inflammation, ishcemia and necrosis of tissue

Question 3

systemic symptoms

Answer 3

fever malaise weight loss fatigue

Question 4

does primary vasculitis have a known cause

Answer 4

no

Question 5

what might secondary vasculitis be triggered by

Answer 5

by an infection, a drug, or a toxin or may occur as part of another inflammatory disorder or cancer

Question 6

is vasculitis an autoimmune conditon

Answer 6

yes

Question 7

what is affected in large and medium vasculitis

Answer 7

endothelial layer due to a foreign pathogen directly attacking it

Question 8

how is the endothelial damaged in small vessel vasculitis

Answer 8

indirectly by healthy cells near it being attacked such as wbc enzymes

Question 9

how does vasculitis porgress

Answer 9

underlying collagen and tissue factor exposed increase blood coagulaiton

Question 10

what happens to the blood vessel walls

Answer 10

they get weaker making aneurysms more liekly and blood flow is reduced which may cause organ ischameia

Question 11

what happens to vessel walls aas they heal

Answer 11

they become harder and stiffer due to fibrin deposits upon healing

Question 12

examples of large vessel vasculitis

Answer 12

giant cell arteritis
takayasus arteritis

Question 13

giant cell arteritis overview

Answer 13

affects branches of carotids
afffects over 50s
rlly high ESR
women more than men affected
arteries are segmentally affected
giant cell embedded in internal elastic lamina on biopsy
causes headaches, visual disturbances, pain while chewing

Question 14

complications of giant cell arteritis if not treated

Answer 14

blindess

Question 15

treatment for most vasculitis

Answer 15

corticosteroids
start 40-60mg prednisolone for large cell

Question 16

takaysus arteritis overview

Answer 16

affects asian women under 40
affects bracnhes off aortic arch
claudication in arms; difference in pulse
can cause a weak non existent pulse, visual and neural symptoms
ESR elevated
similar to giant cell
investigated via ct angiogram

Question 17

examples of medium sized vasculitis overview

Answer 17

polyarteritis nodosa; transmural inflammation - entire vessel wall affected , string of beads on angiogram
middle aged to older
manage via tissue biopsy
cyclophosphamide for treatment along with steroids if organ threatening disease
steroids + Azathioprine/methotrexate/mycophenolate for non organ threatening

Question 18

examples of small vessel vasculitis

Answer 18

granulomatosis with polyangitis- GPA which affects lungs, nasopharynx and kidneys
microscopic polyangitis ; kidneys and lungs affected
churg strauss syndrome
henoch schonlein purpura; IGA MEDIATED

Question 19

investigations for giant cell

Answer 19

ESR/ plasma viscosity and crp raised
ultrasound
pet. ct or angiogram
skip lesions occur so biopsy may be negative

Question 20

which vasculitis is c-anca and p-anca found in

Answer 20

small vessel vasculitis

Question 21

management of GPA and MPA in organ threatening

Answer 21

Induction remission
Glucocorticoids + Rituximab or cyclophosphamamide
Avocopan – C5A receptor inhibitor may be added
?Plasma exchange – when creatinine >300 µmol/L

Maintenance Therapy:
Riuximab or Azathioprine/MTX
Mycophenolate or leflunamide
Resistant disease:

Combination of Rituximab and Cyclophosphamamide
IV immunoglobulin therapy

Question 22

management of non organ threatening disease GPA and MPA

Answer 22

Induction remission
Glucocorticoids + Rituximab or MTX/mycophenolate
Maintenance therapy
Riuximab or Azathioprine/MTX
Mycophenolate or leflunamide

Question 23

does a negative biopsy rule out GCA

Answer 23

no

Question 24

does GCA respond well to steroids

Answer 24

yes

Question 25

which vasculitis is anca negative

Answer 25

medium sized

Question 26

classic feature of polyarteritis nodosa

Answer 26

vessels vary so some may be more damaged than others and some may be at the early stage of activity