Vasculitis Flashcards
What is vasculitis
Group of conditions characterised by inflammatory cell infiltrate and necrosis of BV wall with impairment of blood flow and sometimes damage to vessel wall integrity
Multisystem disease
Symptoms depend on the size and site of the BV involved
Relapsing remitting conditions
One way to categorise them
Vessel size
Primary - large vessel = takaysus and giant cell arteries
Medium - polyarteritis nodosum, Kawasaki syndrome
Med/small - granulomatosis with polyarteritis, microscopic polyarterits, Behçet’s disease
Small - henoch-schonlein purpura, leucocytoplastic vasculitis
Secondary - RA, SLE, Sjogrens
Essential to exclude infecion drugs and malignancy
Which vasculitides are ANCA associated
Granulomatosis with Polyangitis - GPA - Wegners
Microscopic polyangitis -MPA - historically part of polyarteritis nodosum
Eosinophilic granulomatosis with polyangitis - EPA - Churg Straus
What is ANCA what are they against
Anti-neutrophil - cytoplasmic antibody
2 types
pANCA - perinuclearANCA - myeloperoxidase MPO
cANCA -cytoplasmicANCA - PR3 - proteinase 3
Features of granulomatosis with polyangitis
Affects the upper and lower airway Kidney Eye Skin Commonly relapsing Usually a prodrome cANCA PR3
Features of microscopic polyangitis
Affects the kidney, lower airway, eye, skin, PNS Shorter onset less likely to relapse pANCA MPO NO granulomatous formation
Features of eosinophilic granulomatosus polyangitis
Triad Late onset asthma Eosinophilia Vasculitis Affects -peripheral neuropathy - glove and stocking Nasal polyps Myocarditis CNS vasculitis Pulmonary infiltrate 70% ANCA -ve
Immunopathology of ANCA vasculitis
T cells lose there tolerance to PR3 and MPO
B cells produce an ANCA
Neutrophil priming and ANCA activation
ANCA and the neutrophil
Aberrant activation of neutrophils
- endothelial adhesion and migration
- direct tissue injury (superoxide and granulation)
- promotes cytokine release and aberrant apoptosis
Complications of vasculitis
Gangrene or infarct of skin or digits Nasal collapse and subglottic stenosis Pulmonary haemorrhage Coronary arteries Neuropathy Renal failure
Tx of vasculitis
Induce remission and maintain remission Induce steroids Severe - cyclophosphamide, rituximab, plasma exchange Mod/mild = mycophenolate mofetil or methotrexate Maintain Corticosteroids Azathioprine Mycophenolate mofetil Methotrexate Rituximab
