Vasculitis Flashcards
What are the different types of vasculitis?
Small vessel
Medium vessel
Large vessel
What is vasculitis?
Autoimmune inflammation of the blood vessels, most commonly the arteries
How does the inflammation affect the vessel?
It affects the endothelial layer either directly via molecular mimicry or indirectly via attacking cells nearby and the immune effect damages the endothelial layer.
This exposes the collagen and tissue factor causing weakness and increased coagulation. Also become stiffer from fibrin deposits during healing.
Which type of damage occurs in small, medium and large vessel vasculitis?
Small - indirect
Medium and Large - direct
What are symptoms of vasculitis?
Generalised symptoms and then also specific symptoms based on which vessels are affected and which organs become ischaemic.
How does vasculitis cause ischaemia?
Either via blood clots blocking the vessel
OR
Thickening and stiffening of the wall from fibrin deposits causing narrowing
What are the types of large vessel vasculitis?
Giant cell arteritis (temporal, ophthalmic, facial)
Takayasu arteritis
What are the symptoms of giant cell arteritis?
Temporal branch = headaches
Ophthalmic branch = visual disturbances, blindness
Facial branch = jaw claudication
What are risk factors for GCA?
over 50
women
What tests are crucial in GCA?
ESR (really fucking high, over 100)
Fundoscopy
Temporal biopsy - long section due to segmental nature. Negative biopsy doesn’t exclude due to segmental nature.
What are the “giant cells”?
Granulomas which are groups of monocytes packed so tightly that it looks like one giant cell
What is giant cell arteritis?
Segmental vasculitis that affects the branches of the external carotid artery.
What do we do to treat GCA?
Corticosteroids - prednisolone 60mg
What is takayasu arteritis?
It is vasculitis that affects the branches of the aortic arch.
What are the risk factors of takayasu arteritis?
asian woman
under 40
What are the symptoms of takayasu arteritis?
Depending on the artery that is affected:
upper extremities weak, no pulse, cold.
visual and neurological symptoms if carotid affected
How do we treat takayasu arteritis?
corticosteroids, prednisolone 60mg
aspirin 75mg
bone protection (calciferol and/or alendronic acid)
Why do we worry about arteritis?
Can cause occlusion, aneurysm and stenosis.
What are the different types of medium vessel arteritis?
Polyarteritis nodosa
Kawasaki disease
Buerger’s disease (thromboangiitis obliterans)
What are the types of small vessel arteritis?
ANCA associated: Wegeners granulomatosis (GPA) Microscopic polyangitis Churg-Strauss syndrome Eosinophilic granulomatous polyangiitis Pauci-immune Other HSP
What is Kawasaki disease?
Affects mainly children in oriental worlds. It affects the coronary arteries and symptoms include fever, cervical lymphadenopathy.
Treat with intravenous immunoglobulins.
What is polyarteritis nodosa?
Directly attack endothelium, like Hep B. Causes transmural inflammation causing the vascular wall to die, causing fibrinoid necrosis, weakness and bead aneurysms. All vasculitis can also cause livedo reticularis (skin lace)
Which symptoms do people get if the vessels that are affected are the renal arteries?
HTN
nil
Which symptoms do people get if the vessels that are affected are the mesenteric arteries?
abdo pain
gastro bleeding
Which symptoms do people get if the vessels that are affected are the skin arteries?
skin lesions
ulcers
What is Buerger’s disease?
Clots in the vessels supplying the fingers and toes causing necrosis.
What is the biggest risk factor for vasculitis?
Tobacco.
This can trigger the autoimmune response.
What does ANCA actually mean?
Anti neutrophilic cytoplasmic antibodies
This is when the B cell uses IgG to attack neutrophils
What is Wegener’s granulomatosis? (GPA)
(Granulomatosis with polyangitis)
This is a type of ANCA vasculitis called cANCA. These bind to proteinase 3 in neutrophils causing free radicals to damage endothelial cells.
Which parts of the body does GPA affect?
Nasopharynx - saddle nose, sinusitis, ulcers
Lungs - ulcers, constriction
Kidneys - HTN, reduced GFR
How do we treat GPA?
Corticosteroids and cyclophosphamide.
What is microscopic polyangitis (MPA)?
Same as GPA but: Only affects kidney/lungs No granulomas pANCA not cANCA Treat with prednisolone and cyclophosphamide.
What is churg-strauss syndrome?
Same as microscopic polyangiitis (pANCA)
But affects lung, kidney, gastro, skin, nerve and heart. Often mistaken for allergies.
What is HSP?
Henoch-Schonlein Purpura. Own IgA from musocal cells directly targets own endothelial cells.
Commonly causes small bleeds on buttock and legs = palpable purpura, abdo pain from GI affecting, kidney problems = haematuria and IgA nephropathy.
HOW DO WE TREAT VASCULITIS?
Prednisolone 1mg/kg/day
Calciferol and alendronic acid for bone protection
Cyclophosphamide
How does ANCA vasculitis present?
Renal with RPGN
Respiratory with granuloma, cough, dyspnoea, haemoptysis and haemorrhage
ENT with nasal deformity, deafness, rhinorrhoea, crusting and epistaxis
Also in skin, joints, GI, heart and neuro.
What are the specific features of the different kinds of ANCA mediated vasculitis?
GPA: ENT involvement, cANCA, granuloma
EGPA: asthma, eosinophilia, neuropathy, pANCA
MPA: if others are excluded.
What tests do we do if we suspect small cell vasculitis?
Hx and exam ANCA test Kidney biopsy Lung imaging CO transfer factor for haemorrhage of lung (differentiates between pulmonary oedema and blood) Nerve conduction studies ENT assessment
How do we treat ANCA vasculitis?
IV CS
Cyclophosphamide to destroy the immune cells.
Azathioprine and prednisolone (1mg/kg/day) to maintain
MABs
Plasmaphoresis
