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Vasculitis

Introduction
-Inflammation of vessel wall

-Commonly affected small vessels


arterioles to capillaries to venules;
- Some affect particular size/ vessel beds
Classification
Types Examples

Large vessel vasculitis -Giant-cell (Temporal) Arteritis


-Takayasu Arteritis

Medium vessel -Polyarteritis nodosa


vasculitis -Kawasaki disease

Small vessel vasculitis -Wegener granulomatosis


-Churg-Strauss syndrome
-Microscopic polyangiitis
Pathogenesis
-Immune- mediated inflammation

-Direct invasion of vascular walls by infectious pathogens


-Infections can indirectly induce a non-infectious vasculitis
-Eg. generate immune-complexes/ triger cross-reactivity

-Physical & Chemical injury (irradiation, mechanical


trauma, toxin etc)

**Important to differentiate whether it is infectious or non-infectious cause as


Rx. is different. **
Non-infectious v asculitis
1. Immune complex deposition
2. ANCA antibodies
3. Anti-endothelial cell Ab
Non-infectious v asculitis
1. Immune-Complex Assoc. Vasculitis
-Secondary to drug hypersensitivity
-Ab directed against drug-modified protein/ foreign molecules
>> immune complexes formation
-C/f frequently seen in skin
-Drugs bind to serum protein (eg.penicillin)
-Drugs themselves as foreign protein (eg. streptokinase)
-** discontinuation of drug lead to resolution

-Secondary to viral infection


-Ab to viral proteins >> immune complexes formed & found in
serum & vascular lesion
-Eg. Polyarteritis nodosa
Non-infectious vasculitis
2. ANCA (Anti-neutrophil Cytoplasmic Ab)
-Presence of circulating Ab (ANCA) react with neutrophil cytoplasmic Ag

-2 types: MPO-ANCA / PR3-ANCA

-ANCA directly activate neutrophils >> stimulate neutrophils release


reactive O2 species & proteolytic enzymes >> endothelial cell-neutrophil
interaction >> endothelial cell damage

-Mechanism of ANCA vasculitis


-Drug/ cross-reactivity microbial Ag induce ANCA
-Subsequent infection, endotoxin exposure
-Cytokines (eg.TNF) >> surface expression of MPO/ PR3 on neutrophils
(cytokine-activated cells) >> direct injury/ induce further activation
-ANCA activated neutrophils degranulate >> release ROS >> injury &
engendering endothelial cell toxicity
Non-infectious vasculitis
3. Anti-Endothelial Cell Ab
- Ab to endothelial cells (eg. Kawasaki dx)
Infectious Vasculitis
-Localised arteritis, d/t
-Direct invasion of infectious agents (usually
bac./fungi-Aspergillus & Mucor sp.)
-Localised tissue infection >> vascular invasion (eg.
bac.pneumonia/ adjacent to abscess/ hematogenous
seeding of bac. during septicaemia/ embolization from
sepsis of IE)

-Vascular infection >> weaken arterial walls >>


mycotic aneurysm/ induce thrombosis & infarction