Vasculidities Flashcards

Large, Medium and Small Vessel Vasculidities (42 cards)

1
Q

What is meant by the term vasculitis?

A

Inflammation of blood vessels

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2
Q

What are the three categories of vasculitis?

A

Small, medium and large vessel

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3
Q

List 4 types of small vessel vasculitis

A
  • henoch-schonlein purpura
  • microscopic polyangiitis
  • granulomatosis with polyangiitis (Wegner’s)
  • eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
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4
Q

List 3 types of medium vessel vasculitis

A
  • polyarteritis nodosa
  • Kawasaki disease
  • Buerger’s disease
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5
Q

List 2 types of large vessel vasculitis

A
  • giant cell arteritis
  • Takayasu’s arteritis
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6
Q

Describe what happens in the body to cause vasculitis

A

Usually an auto-immune response where WBC’s attack the endothelial layer of blood vessels

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7
Q

What happens when endothelium is damaged in blood vessel walls?

A

Collagen and tissue factor are exposed, which leads to coagulation, and therefore an increased risk of stroke

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8
Q

Why does vasculitis increase the risk of aneurisms?

A

Fibrin is deposited in the vessel walls at an attempt to repair them, however this makes the walls less stretchy, so more vulnerable to aneurisms

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9
Q

List some systemic symptoms that may be seen in vasculitis

A

Symptoms related to inflammation such as fever, weight loss and fatigue

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10
Q

What blood vessels tend to be affected by giant cell arteritis?

A

Branches of the carotid arteries, e.g., the temporal, ophthalmic and facial arteries

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11
Q

List some symptoms of giant cell arteritis

A

headaches
visual disturbances
jaw claudication

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12
Q

What is seen on investigations for giant cell arteritis?

A

bloods show raised ESR
temporal artery biopsy shows giant cells in the elastic lamina

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13
Q

What is the treatment for giant cell arteritis?

A

Corticosteroids - high dose prednisolone
Dose depends on whether or not the patient has visual disturbances

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14
Q

What blood vessels are affected in Takayasu’s arteritis?

A

Branches of the aortic arch, that supply the upper extremities and head

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15
Q

List some symptoms of Takayasu’s arteritis

A

weak/no pulse
neurological symptoms
muscle aches

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16
Q

What is seen on investigations for Takayasu’s arteritis?

A

bloods show raised ESR
giant cells seen in elastic lamina
CT/MRI angiography helpful for diagnosis, instead of a biopsy

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17
Q

What blood vessels are affected by Kawasaki disease?

A

Coronary arteries

18
Q

What demographic usually gets Kawasaki disease?

A

children under 5
more common in boys than girls

19
Q

What is the CRASH + burn criteria for Kawasaki disease?

A

diagnosis can be made with 4/5 of the following:
C - conjunctivitis
R - rash that peels later on
A - adenopathy
S - strawberry tongue
H - hands and feet swollen
+ fever for 5 or more days

20
Q

What is seen on investigations for Kawasaki disease?

A

bloods show anaemia and raised WBCs
blood film shows increase in immature WBCs
raised CRP and ESR
raised liver enzymes in late stage
echocardiogram used for diagnosis

21
Q

How is Kawasaki disease treated?

A

IV Ig’s
aspirin - note that this is usually not given to children, but the benefits outweigh the risks in this case

22
Q

What can cause polyarteritis nodosa?

A

infections/vaccines - key examples include strep, staph and hep B

23
Q

Describe what happens to blood vessels in polyarteritis nodosa

A

all layers of the vessel wall are damaged (transmural disease)
angiogram shows a ‘string of beads’ which are multiple aneurisms

24
Q

What symptoms may be seen in polyarteritis nodosa?

A

symptoms depend on affected vessels
- renal ischaemia can cause hypertension
- mesenteric ischaemia can cause abdo pain and GI bleeding
- neuro ischaemia can cause neurological symptoms
- skin is often affected, presenting as livedo reticularis

25
What demographic usually gets Buerger's disease?
men between 20-40 increased risk in tobacco users
26
What happens in Buerger's disease and what symptoms does this cause?
clots form in blood vessels supplying digits this leads to ulcers, necrosis and autoamputation
27
What is happening in the immune system in small vessel vasculidities?
B cell antibodies (mainly IgG) attack granules made by neutrophils
28
What is the name for the antibodies that cause damage in small vessel vasculidities?
anti-neutrophilic cytoplasmic antibodies (ANCA's)
29
What is the name for the antibodies that cause damage in granulomatosis with polyangiitis?
cytoplasmic anti-neutrophilic cytoplasmic antibodies (c-ANCA's)
30
What do c-ANCA's bind to?
proteinase-3
31
What symptoms are seen in granulomatosis with polyangiitis?
nasopharynx symptoms - pain, bloody mucus, saddle-nose respiratory symptoms - haemoptysis, difficulty breathing renal symptoms - reduced urine production, raised BP
32
How is granulomatosis with polyangiitis and microscopic polyangiitis treated?
corticosteroids cyclophosphamide (immunosuppressant chemotherapy agent)
33
What is the name for the antibodies that cause damage in microscopic polyangiitis?
perinuclear anti-neutrophilic cytoplasmic antibodies (p-ANCA's)
34
What are the 2 main differences between granulomatosis with polyangiitis and microscopic polyangiitis?
in MP, there are no granulomas and no effect on the nasopharynx
35
What do p-ANCA's bind to?
myeloperoxidase
36
What symptoms are seen in eosinophilic granulomatosis with polyangiitis?
sinusitis - commonly mistaken for allergies or asthma lung damage kidney damage GI, skin, nerve and heart damage can also be seen
37
What is seen on investigations in eosinophilic granulomatosis with polyangiitis?
raised eosinophils p-ANCA or sometimes c-ANCA granulomas can form
38
What is seen on investigations in Henoch-Schonlein purpura?
no ANCA raised IgA - found in mucosal cells
39
What symptoms can be seen in Henoch-Schonlein purpura?
palpable purpuric rash, common around buttocks and legs HTN, GI pain, neurological symptoms, depending on organs affected
40
In which vasculidities are the vessel walls directly attacked?
medium and large vasculidities Henoch-Schonlein purpura
41
In which vasculidities are the vessel walls indirectly attacked?
small vessel vasculidities, with the exception of Henoch-Schonlein purpura
42
What are skip lesions and what is their clinical relevance?
areas of the blood vessel wall that appear normal on a biopsy this is important, as it means that a temporal artery biopsy CANNOT rule out GCA