Vasculitis Flashcards
What is vasculitis?
Inflammation of blood vessels that may result in vessel wall thickening, stenosis and occlusion with subsequent ischaemia.
Define large vessel vasculitis
Primary vasculitis causing chronic granulomatous inflammation of the aorta and its branches.
What are the types of large vessel vasculitis?
Temporal (giant cell) arteritis
Takayasu arteritis (TA)
Rarely occur before 50 y.o.
Who does Takayasu arteritis usually affect?
Young women 20s-30s
How does large vessel vasculitis present?
Low-grade fever Malaise Night sweats Weight loss Arthralgia Fatigue Claudicant symptoms in both upper and lower limbs Untreated --> vascular stenosis and aneurysms (resulting in pulses and bruits)
Investigations into large vessel vasculitis
ESR, PV and CRP elevation
MR angioplasty shows thickened vessel walls and stenosis
PET CT shows increased metabolic activity in the large vessels
Treatment of large vessel vasculitis
Prednisolone 40-60mg
Gradually reduce dose
Steroid-sparing agents such as methotrexate and azathioprine may be added.
How is small to medium vessel vasculitis divided up?
Into ANCA positive and negative conditions
How does small to medium vessel vasculitis present?
Fever Weight loss Raised, non-blanching, purpuric rash Arthralgia/arthritis Mononeuritis multiplex Glomerulonephritis Lung opacities on x-ray
What are the 4 types of vasculitis associated with ANCA
Granulomatosis with polyangiitis (Wegener’s; GPA)
Microscopic polyangiitis (MPA)
Renal limited vasculitis (RLV)
Chrug-Strauss syndrome (CSS)
What does Wegener’s vasculitis present with?
ENT symptoms (nose bleeds, recurrent sinusitis, nasal crusting)
Haemoptysis
Cavitating lesions on x-ray
cANCA and PR3 (proteinase-3) association
What does microscopic polyangiitis present with?
Glomerulonephritis in up to 90% of patients Raised ESR, PV and CRO Anaemia U and E ANCA Renal vasculitis
Investigations in ANCA positive vasculitis’
Raised ESR, PV and CRO Anaemia U and E for renal involvement ANCA Renal vasculitis - urinalysis CXR Biopsy of affected area
Management of ANCA positive vasculitis’
IV steroids and cyclophosphamide due to aggressive disease course
What is Henoch-Schonlein purpura?
An acute immunoglobulin A (IgA) mediated disorder characterised by a generalised vasculitis involving the small vessels of the skin, GI tract, kidneys, joints , lungs and CNS
