Vasculitis

Question 1

What is vasculitis?

Answer 1

Inflammation of blood vessels

Question 2

How can vasculitis be classified? (2)

Answer 2

• Size of vessel
• ANCA positive or negative

Question 3

What are the two major categories of large vessel vasculitis?

Answer 3

1. Giant cell arteritis
2. Takayasu arteritis

Question 4

Which arteries are most common for giant cell arteritis to affect?

Answer 4

• Temporal artery (most common)
• Opthalmic artery
• Facial artery

Question 5

The endothelium is generally directly attacked with molecular mimicry in which two classes of vasculitis?

Answer 5

• Large vessel
• Medium vessel

Question 6

The endothelium is generally indirectly attacked in which class of vasculitis?

Answer 6

Small vessel

(N.b Henloch-Scholein purpura is an exeption to this rule)

Question 7

What happens to the vessel walls due to the inflammation is vasculitis?

Answer 7

• Inflammatory processes cause tissue factor and collagen to be exposed (endothelial cells die)
• Thrombosis occurs
• Vessel walls weak leading to aneurysm
• Fibrosis due to fibrin deposition occurs as part of the healing process
• Both thrombosis and fibrosis narrow the vessel lumen reducing blood flow

Question 8

Ultimately, vasculitis leads to less ______ _____

Answer 8

Bloof flow

Question 9

Why does jaw claudication occur with giant cell arteritis?

Answer 9

The facial artery is affected and less blood flow to the face occurs

Question 10

Giant cell arteritis is most common in what age group and sex?

Answer 10

Women > 50

Question 11

What is found in the elastic lamina in a patient with giant cell arteritis?

Answer 11

Granulomas or “giant cells”

Question 12

When taking a biopsy for giant cell arteritis, why is a negative result not able to rule out the condition?

Answer 12

Giant cell arteritis is segmental

Question 13

Takayasu arteritis is most common in which age group and sex?

Answer 13

Asian women < 40

Question 14

Takayasu arteritis generally affects which arteries?

Answer 14

Those off of the aortic arch

Question 15

In patients with Takayasu arteritis, what is found in the internal elastic lamina?

Answer 15

Granulomas or “giant cells”

Question 16

What are some common medium vessel arteritides?

Answer 16

1. Kawasaki disease
2. Polyarteritis nodosa

Question 17

Kawasaki disease generally affects people of what age?

Answer 17

< 5

(boys more than girls)

Question 18

What are the symptoms of Kawasaki disease?

Answer 18

Conjuctivitis (sparing the limbus)

Rash over body

Adenopathy (large or swollen lymph nodes)

Strawberry tongue

Hands and feet swelling

Fever

(Patients crash and burn)

Question 19

Kawasaki disease is usually _____ __________

Answer 19

Self limiting

Question 20

How can Kawasaki disease be treated?

Answer 20

IV Ig

Aspirin (monitor very closely for Reye syndrome)

Question 21

What is the pathogenesis behind polyarteritis nodosa?

Answer 21

Immune cells directly attack the endothelial cells

This is an example of molecular mimicry as the antibodies produced are designed to takle hepatitis B

Question 22

Polyarteritis nodosa causes ___________ necrosis

Answer 22

Transmural

Question 23

Fibrinoid necrosis occurs with polyarteritis nodosa which leaves the vessels prone to what?

Answer 23

Aneurysm

Question 24

Upon angiogram, polyarteritis nodosa has a very distinctive appearance. What is this appearance?

Answer 24

“String of beads”

Question 25

In polyarteritis nodosa what happens when renal arteries are affected?

Answer 25

Hypertension (low glomerular pressure leads to fluid retention and increased blood pressure)

Question 26

What can occur when the mesenteric arteries are affected in polyarteritis nodosa?

Answer 26

* Abdominal pain * GI bleed

Question 27

What is ANCA?

Answer 27

Anti-neutrophil cytoplasmic antibody

Question 28

Wegener's granulomatosis (Granulomatosis with polyangiitis) involves which type of ANCA?

Answer 28

cANCA | (cytoplasmic ANCA)

Question 29

In Wegener's granulomatosis, cANCA binds to what?

Answer 29

Proteinase 3 within the neutrophilic membrane

Question 30

When pANCA or cANCA binds to neutrophils, why does the endothelium end up damaged?

Answer 30

O₂ free radicals are released from neutrophils causing indirect damage

Question 31

Name some small vessel vasculitides?

Answer 31

* Wegener's granulomatosis * Microscopic polyangiitis * Churg-Strauss syndrome * Henloch-Schonlein purpura * Renal limited vasculitis

Question 32

Wegener's granulomatosis is more common in which sex?

Answer 32

Men

Question 33

Which areas of the body does Wegener's granulomatosis commonly affect?

Answer 33

Nasopharynx (painful sinusitis, bloody mucous from ulcers and saddle nose deformity) Lungs (difficulty breathign due to ulcer formation and bloody cough) Kidneys

Question 34

Saddle nose deformity is associated with which vasculitis?

Answer 34

Wegener's granulomatosis

Question 35

Once cured, relapse of Wegener's granulomatosis is what?

Answer 35

Common

Question 36

How is microscopic polyangiitis different from Wegener's granulomatosis?

Answer 36

1. Does not affect nasopharynx (only kidneys and lungs) 2. No granulomas can be found in vessle walls 3. pANCA vs cANCA

Question 37

What is pANCA?

Answer 37

Perinuclear ANCA

Question 38

After microscopic polyangiitis is cured or overcome, relapse is what?

Answer 38

Common

Question 39

Churg-Strauss syndrome is commonly mistaken for what?

Answer 39

Asthma or allergies

Question 40

Which immune cell type is particularly elevated in Churg-Strauss syndrome?

Answer 40

Eosinophils

Question 41

Which areas of the body does Churg-Strauss syndrome affect?

Answer 41

* Sinuses * Lungs * Kidneys * GI tract * Skin * Nerves * Heart

Question 42

Are granulomas associated with Churg-Strauss syndrome?

Answer 42

Yes

Question 43

What makes Henloch-Schonlein purpura different from other small vessel vasculitides?

Answer 43

There are no ANCAs involved

Question 44

What causes Henloch-Schonlein purpura?

Answer 44

IgA targets endothelial cells via molecular mimicry

Question 45

The purpura seen in Henloch-Schonlein purpura is distinctive, but why?

Answer 45

It is palpable This is due to the fibrosis occurng in the affected vessels

Question 46

Henloch-Schonlein purpura can affect two other major body systems besides the skin, what are these?

Answer 46

GI (abdominal pain) Renal (haematuria, IgA nephropathy)

Question 47

Henloch-Schonlein purpura can resolve on its own. True or false?

Answer 47

True

Question 48

Large vessel vasculitis can lead to reduced _______ and carotid \_\_\_\_\_\_\_\_

Answer 48

Pulses Bruit

Question 49

What is the treatment for large vessle vasculitis?

Answer 49

40-60mg of prednisolone which granually reduces

Question 50

What is the most common complication of microscopic polyangiitis?

Answer 50

Glomerulonephritis

Question 51

Most cases of ANCA +ve vasculitis require what treatment?

Answer 51

IV steroids Cyclophosphamide

Question 52

Henloch-Schonlein purpura commonly affects children and a history involving what is common?

Answer 52

An upper respiratory tract infection predating symptom onset by a few weeks

Question 53

Henloch-Schonlein purpura has what symptoms commonly?

Answer 53

* Purpuric rash over buttocks and lower limbs * Abdominal pain * Vomiting * Joint pain

Question 54

Henloch-Schonlein purpura requires what treatment?

Answer 54

It is a self limiting condition which does nto require much specific treatment It will settle over a period of weeks to months

Question 55

Temporal arteritis has a common association with what?

Answer 55

Polymyalgia rheumatica

Question 56

cANCA and anti-PR3 are asscoiated with what?

Answer 56

Wegener's granulomatosis

Question 57

pANCA and anti-MPO are associated with what?

Answer 57

Microscopic polyangiitis

Question 58

Anti-MPO or anti-PR3 are asscoiated with what?

Answer 58

Churg-Strauss syndrome (Eosinophilic granulomatosis with polyangiitis)