Vasculitis Flashcards

1
Q

What are the different types of vasculitis?

A

Small vessel
Medium vessel
Large vessel

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2
Q

What is vasculitis?

A

Autoimmune inflammation of the blood vessels, most commonly the arteries

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3
Q

How does the inflammation affect the vessel?

A

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.

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4
Q

Which type of damage occurs in small, medium and large vessel vasculitis?

A

Small - indirect

Medium and Large - direct

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5
Q

What are symptoms of vasculitis?

A

Generalised symptoms and then also specific symptoms based on which vessels are affected and which organs become ischaemic.

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6
Q

How does vasculitis cause ischaemia?

A

Either via blood clots blocking the vessel
OR
Thickening and stiffening of the wall from fibrin deposits causing narrowing

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7
Q

What are the types of large vessel vasculitis?

A

Giant cell arteritis (temporal, ophthalmic, facial)

Takayasu arteritis

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8
Q

What are the symptoms of giant cell arteritis?

A

Temporal branch = headaches
Ophthalmic branch = visual disturbances, blindness
Facial branch = jaw claudication

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9
Q

What are risk factors for GCA?

A

over 50

women

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10
Q

What tests are crucial in GCA?

A

ESR (really fucking high, over 100)
Fundoscopy
Temporal biopsy - long section due to segmental nature. Negative biopsy doesn’t exclude due to segmental nature.

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11
Q

What are the “giant cells”?

A

Granulomas which are groups of monocytes packed so tightly that it looks like one giant cell

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12
Q

What is giant cell arteritis?

A

Segmental vasculitis that affects the branches of the external carotid artery.

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13
Q

What do we do to treat GCA?

A

Corticosteroids - prednisolone 60mg

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14
Q

What is takayasu arteritis?

A

It is vasculitis that affects the branches of the aortic arch.

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15
Q

What are the risk factors of takayasu arteritis?

A

asian woman

under 40

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16
Q

What are the symptoms of takayasu arteritis?

A

Depending on the artery that is affected:
upper extremities weak, no pulse, cold.
visual and neurological symptoms if carotid affected

17
Q

How do we treat takayasu arteritis?

A

corticosteroids, prednisolone 60mg
aspirin 75mg
bone protection (calciferol and/or alendronic acid)

18
Q

Why do we worry about arteritis?

A

Can cause occlusion, aneurysm and stenosis.

19
Q

What are the different types of medium vessel arteritis?

A

Polyarteritis nodosa
Kawasaki disease
Buerger’s disease (thromboangiitis obliterans)

20
Q

What are the types of small vessel arteritis?

A
ANCA associated:
Wegeners granulomatosis (GPA)
Microscopic polyangitis
Churg-Strauss syndrome
Eosinophilic granulomatous polyangiitis
Pauci-immune 
Other
HSP
21
Q

What is Kawasaki disease?

A

Affects mainly children in oriental worlds. It affects the coronary arteries and symptoms include fever, cervical lymphadenopathy.
Treat with intravenous immunoglobulins.

22
Q

What is polyarteritis nodosa?

A

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)

23
Q

Which symptoms do people get if the vessels that are affected are the renal arteries?

A

HTN

nil

24
Q

Which symptoms do people get if the vessels that are affected are the mesenteric arteries?

A

abdo pain

gastro bleeding

25
Q

Which symptoms do people get if the vessels that are affected are the skin arteries?

A

skin lesions

ulcers

26
Q

What is Buerger’s disease?

A

Clots in the vessels supplying the fingers and toes causing necrosis.

27
Q

What is the biggest risk factor for vasculitis?

A

Tobacco.

This can trigger the autoimmune response.

28
Q

What does ANCA actually mean?

A

Anti neutrophilic cytoplasmic antibodies

This is when the B cell uses IgG to attack neutrophils

29
Q

What is Wegener’s granulomatosis? (GPA)

A

(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.

30
Q

Which parts of the body does GPA affect?

A

Nasopharynx - saddle nose, sinusitis, ulcers
Lungs - ulcers, constriction
Kidneys - HTN, reduced GFR

31
Q

How do we treat GPA?

A

Corticosteroids and cyclophosphamide.

32
Q

What is microscopic polyangitis (MPA)?

A
Same as GPA but:
Only affects kidney/lungs
No granulomas
pANCA not cANCA
Treat with prednisolone and cyclophosphamide.
33
Q

What is churg-strauss syndrome?

A

Same as microscopic polyangiitis (pANCA)

But affects lung, kidney, gastro, skin, nerve and heart. Often mistaken for allergies.

34
Q

What is HSP?

A

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.

35
Q

HOW DO WE TREAT VASCULITIS?

A

Prednisolone 1mg/kg/day
Calciferol and alendronic acid for bone protection
Cyclophosphamide

36
Q

How does ANCA vasculitis present?

A

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.

37
Q

What are the specific features of the different kinds of ANCA mediated vasculitis?

A

GPA: ENT involvement, cANCA, granuloma

EGPA: asthma, eosinophilia, neuropathy, pANCA

MPA: if others are excluded.

38
Q

What tests do we do if we suspect small cell vasculitis?

A
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
39
Q

How do we treat ANCA vasculitis?

A

IV CS
Cyclophosphamide to destroy the immune cells.

Azathioprine and prednisolone (1mg/kg/day) to maintain

MABs
Plasmaphoresis