Vasculitis Flashcards
What is vasculitis?
Inflammation of blood vessels
What happens to the blood vessels in vasculitis?
Necrosis
Destruction of vessel walls leading to perforation and haemorrhage into tissues
Endothelial injury leading to thrombosis and ischaemia of tissue
What cells are involved in the inflammatory process?
Vessel wall infiltration by:
- neutrophils
- mononuclear cells
- giant cells
What would you see under a microscope looking at a biopsy of a vessel affected by vasculitis?
Neutrophils
Mononuclear cells
Fibrinoid necrosis
Leukocytoclasis
What is fibrinoid necrosis?
Necrosis of tissue in which there is accumulation of amorphous, basic, proteinaceous material in the tissue matrix with a staining pattern reminiscent of fibrin.
What is leukocytoclasis?
The damage cause by nuclear debris from infiltrating neutrophils
What are some vasculitides that affect large arteries?
Giant cell arteritis
Takyasu’s arteritis
What are some vasculitides that affect medium arteries?
Polyarteritis nodosa
Kawasaki disease
What are some vasculitides that affect small arteries?
Wegener’s granulomatosis
Churg Strauss
What are some vasculitides that affect small vessels?
Henoch Schonlein purpura
What are some secondary causes of vasculitis?
Infection
Drugs
Malignancy
What is ANCA?
Anti-neutrophil cytoplasmic antibodies
Specific for the antigens found in cytoplasmic granules of neutrophils and monocytes
Attack the body’s own cells = autoimmune
What conditions must you exclude when considering a diagnosis of vasculitis?
Sepsis
Malignancy
Cholesterol Emboli
What are some other names for giant cell arteritis?
Temporal arteritis
Cranial arteritis
What is GC arteritis?
Granulomatous thickening of the inner portions of large vessels:
- aorta
- extra-cranial branches of carotid
This causes inflammation of the vessels and pain
What are the branches of the extra-cranial artery that can be affected in GC arteritis?
Ophthalmic artery
Temporal artery
Who gets GC arteritis?
Almost all patients are over 50
Incidence increases with age
Twice as common in women as in men
Clinical features of GC arteritis?
Headache
Temporal + scalp tenderness (brushing hair is painful)
Jaw claudication
Amaurosis fugax
Sudden, painless blindness in 1 eye
Non-specific malaise
Polymyalgia rheumatica
Temporal arteries: tender, palpable, reduced pulsation
What is amaurosis fugax?
Transient visual loss
Looks like a curtain coming down in front of the eyes
It often occurs a few times before full blindness occurs
What is polymyalgia rheumatica?
Pain in many joints, especially shoulder, neck, hips
What would you see examining the temporal artery of someone with GC arteritis?
Palpable
Tender
Reduced pulsation
Investigation of GC arteritis?
Temporal artery biopsy: you will see evidence of inflammation
Bloods: raised ESR and CRP
Look at their optic disc:
- pale
- swollen
- flame shaped haemorrhages at margin
Treatment of GC arteritis?
Start corticosteroids immediately! to prevent blindness
Continue steroids but gradually reduce them over time
Consider non-steroids: methotrexate
Prophylaxis for steroid induced osteoporosis
Time is of the essence in GC arteritis, why?
Because it can lead to permanent bilateral blindness if not treated quickly enough
What is Takayasu’s arteritis?
Vasculitis of aortic arch and other major arteries
Clinical features of Takayasu’s arteritis?
Hypertension, strokes, heart failure, absent peripheral pulse
Management of Takayasu’s arteritis?
Corticosteroids
What is polyarteritis nodosa?
Necrotising vasculitis affecting medium sized vessels, especially renal and visceral ones
It causes aneurysms and thrombosis in these vessels
Who gets polyarteritis nodosa?
Middle age men
What is the link between Hep B and polyarteritis nodosa?
Presence of Hep B antigens in the blood is linked with polyarteritis nodosa.
It is thought that the immune complexes (antigen-antibody) are deposited in places they shouldn’t be causing an inflammatory response
Clinical features of polyarteritis nodosa?
Fever
Malaise
Weight loss
Skin: rash and punched out ulcers
Renal, cardiac, GI and GU involvement
Investigation of polyarteritis nodosa?
Blood:
- raised ESR + CRP
- raised WBC
- anaemia
Angiography: of renal and mesenteric areas to look at blood flow
Renal biopsy
Management of polyarteritis nodosa?
Control BP meticulously
Corticosteroids + immunosuppressants
If linked with Hep B, treat this
There are 2 types of small vessel vasculitis. What are they?
ANCA positive
ANCA negative
Name some small vessel vasculitides which are:
- ANCA positive
- ANCA negative?
ANCA +ve
- GPA aka Wegener’s granulomatosis
- Microscopic polyangiitis
ANCA -ve
- Henoch Schonlein purpura
- Goodpasture’s syndrome
What is Granulomatosis with Polyangiitis (GPA) also known as?
Wegener’s granulomatosis
What is GPA?
Necrotising, granulomatous vasculitis of small vessels all over the body:
- arterioles
- capillaries
- post capillary venules
Is GPA ANCA negative or positive?
ANCA positive
Who gets GPA?
Any age, but peaks between 25-60
Clinical features of GPA?
Affects vasculature of all organ systems
Saddle nose deformity Eye proptosis (eye pushed outwards) Many more
Treatment of GPA?
Steroids
Biologics
DMARDs
Monitor for organ damage
Investigations of GPA?
Blood:
- raised ESR + CRP
- anaemia
Urine:
- protein and haematuria
CXR: shows lung lesions
