vasculitis Flashcards
what is vasculitis
inflammation of blood vessels; arteries, arterioles, veins, venules or capillaries
what can vasculitis lead to
inflammation, ishcemia and necrosis of tissue
systemic symptoms
fever malaise weight loss fatigue
does primary vasculitis have a known cause
no
what might secondary vasculitis be triggered by
by an infection, a drug, or a toxin or may occur as part of another inflammatory disorder or cancer
is vasculitis an autoimmune conditon
yes
what is affected in large and medium vasculitis
endothelial layer due to a foreign pathogen directly attacking it
how is the endothelial damaged in small vessel vasculitis
indirectly by healthy cells near it being attacked such as wbc enzymes
how does vasculitis porgress
underlying collagen and tissue factor exposed increase blood coagulaiton
what happens to the blood vessel walls
they get weaker making aneurysms more liekly and blood flow is reduced which may cause organ ischameia
what happens to vessel walls aas they heal
they become harder and stiffer due to fibrin deposits upon healing
examples of large vessel vasculitis
giant cell arteritis
takayasus arteritis
giant cell arteritis overview
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
complications of giant cell arteritis if not treated
blindess
treatment for most vasculitis
corticosteroids
start 40-60mg prednisolone for large cell
takaysus arteritis overview
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
examples of medium sized vasculitis overview
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
examples of small vessel vasculitis
granulomatosis with polyangitis- GPA which affects lungs, nasopharynx and kidneys
microscopic polyangitis ; kidneys and lungs affected
churg strauss syndrome
henoch schonlein purpura; IGA MEDIATED
investigations for giant cell
ESR/ plasma viscosity and crp raised
ultrasound
pet. ct or angiogram
skip lesions occur so biopsy may be negative
which vasculitis is c-anca and p-anca found in
small vessel vasculitis
management of GPA and MPA in organ threatening
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
management of non organ threatening disease GPA and MPA
Induction remission
Glucocorticoids + Rituximab or MTX/mycophenolate
Maintenance therapy
Riuximab or Azathioprine/MTX
Mycophenolate or leflunamide
does a negative biopsy rule out GCA
no
does GCA respond well to steroids
yes
which vasculitis is anca negative
medium sized
classic feature of polyarteritis nodosa
vessels vary so some may be more damaged than others and some may be at the early stage of activity
