Vasculitis

Question 1

What is vasculitis? Does is mainly affect arteries or veins?

Answer 1

• inflammation of the blood vessel wall

- mainly affects arteries

Question 2

What are the three broad categories of vasculitis?

Answer 2

• large, medium, and small vessel vasculitis

- (there is considerable overlap)

Question 3

What are the two main types of large vessel vasculitis?

Answer 3

• temporal (giant cell) arteritis

- Takayasu arteritis

Question 4

What are the three main types of medium vessel vasculitis?

Answer 4

• polyarteritis nordosa
• Kawasaki disease
• Buerger’s disease

Question 5

What are the four main types of small vessel vasculitis?

Answer 5

• Wegener granulomatosis
• microscopic polyangiitis
• Churg-Strauss syndrome
• HSP (Henoch-Schonlein purpura)

Question 6

Temporal (Giant Cell) Arteritis is most common in what demographic? What about Takayasu Arteritis?

Answer 6

• giant cell: female patients > 50

- Takayasu: patients

Question 7

What type of vasculitis is Temporal (Giant Cell) Arteritis? Which vessels does it commonly affect?

Answer 7

• large vessel vasculitis

- commonly affects the carotid artery

Question 8

Why do patients with Temporal (Giant Cell) Arteritis commonly present with headaches?

Answer 8

• because the temporal artery branches from the carotid, and the inflammed artery causes pressure in the head

Question 9

What is a major potential risk of Temporal (Giant Cell) Arteritis?

Answer 9

• blindness due to the proximity of the ophthalmic artery to the carotid

Question 10

Pathophysiology of Temporal (Giant Cell) Arteritis; What do we see on histology?

Answer 10

• giant cells attack and fragment the internal elastic lamina of the blood vessel wall
• on histology, we would see a huge separation between the intima and media as a result

Question 11

What type of vasculitis is Takayasu Arteritis? Which vessels does it commonly affect?

Answer 11

• large vessel vasculitis

- commonly affects the aortic arch at its branch points

Question 12

Pathophysiology of Takayasu Arteritis

Answer 12

• essentially the same as temporal arteritis

- giant cells attack and fragment the internal elastic lamina of the blood vessel wall

Question 13

Medium vessel vasculitis involves which vessels?

Answer 13

• muscular arteries that supply organs

Question 14

Polyarteritis Nordosa

Answer 14

• a necrotizing (fibrinoid necrosis) arteritis that is systemic (affects most organs)
• a medium vessel vasculitis

Question 15

Polyarteritis Nordosa is most common in which demographic?

Answer 15

• young adults

Question 16

What type of inflammation is involved in Temporal Arteritis and Takayasu Arteritis?

Answer 16

• granulomatous inflammation (hence the giant cells)

Question 17

What type of necrosis is involved in Polyarteritis Nordosa?

Answer 17

• fibrinoid necrosis

Question 18

Although it is systemic, which organs are NOT affected by Polyarteritis Nordosa?

Answer 18

• the lungs

Question 19

What do we see on histology of Polyarteritis Nordosa?

Answer 19

• a ‘string-of-pearls’ appearance

Question 20

What type of vasculitis is Kawasaki Disease? Which vessels does it usually affect?

Answer 20

• medium vessel vasculitis

- affects the coronary artery

Question 21

Kawasaki Disease is most common in which demographic?

Answer 21

• asian children

Question 22

Kawasaki disease commonly affects the coronary artery, greatly increasing the risk of ___________, despite the patient’s young age. Therefore, we commonly give these patients prophylactic ________.

Answer 22

• increased of MI

- give them aspirin!

Question 23

What type of vasculitis is Buerger’s Disease? Which vessels does it affect?

Answer 23

• a necrotizing medium vessel vasculitis

- affects the arteries in the digits

Question 24

Buerger’s Disease is only seen in which demographic?

Answer 24

• smokers!

- it is totally associated with smoking

Question 25

Patients with Buerger's Disease commonly present with what symptoms?

Answer 25

- ulceration and gangrene of their fingers and toes | - if untreated, autoamputation can occur

Question 26

Small vessel vasculitis affects which vessels?

Answer 26

- arterioles, capillaries, and venules

Question 27

What type of vasculitis is Wegener Granulomatosis?

Answer 27

- a necrotizing small vessel vasculitis

Question 28

What is the classic triad of affected organs in Wegener Granulomatosis?

Answer 28

- the nasopharynx, lungs, and kidneys

Question 29

Wegener Granulomatosis is most common in which demographic?

Answer 29

- middle aged males

Question 30

Small vessel vasculitis is commonly ANCA related. Is Wegener Granulomatosis ANCA related? If so, what type?

Answer 30

- (anti-neutophril cytoplasmic antibodies) | - Wegener granulomatosis is c-ANCA related

Question 31

What type of vasculitis is Microscopic Polyangiitis?

Answer 31

- a necrotizing small vessel vasculitis

Question 32

Which organs are commonly affected in Microscopic Polyangiitis?

Answer 32

- multiple organs, but especially the skin, lungs, and kidney

Question 33

Microscopic Polyangiitis and Wegener Granulomatosis are both types of necrotizing small vessel vasculitis that affect the kidneys and lungs - how can we tell them apart?

Answer 33

- microscopic polyangiitis does NOT affect the nasopharynx, and is p-ANCA related - Wegener granulomatosis DOES affect the nasopharynx, and is c-ANCA related

Question 34

Small vessel vasculitis is commonly ANCA related. Is Microscopic Polyangiitis ANCA related? If so, what type?

Answer 34

- (anti-neutrophil cytoplasmic antibodies) | - microscopic polyangiitis is p-ANCA related

Question 35

Churg-Strauss Syndrome is a type of which vasculitis? Which organs are commonly affected?

Answer 35

- a rare necrotizing small vessel vasculitis with eosinophils - several organs affected, but especially the lungs and heart

Question 36

Small vessel vasculitis is commonly ANCA related. Is Churg-Strauss Syndrome ANCA related? If so, what type?

Answer 36

- (anti-neutrophil cytoplasmic antibodies) | - Churg-Strauss syndrome is p-ANCA related

Question 37

Miscroscopic Polyangiitis and Churg-Strauss Syndrome are both types of necrotizing small vessel vasculitis that are p-ANCA related - how can we tell them apart?

Answer 37

- eosinophils are only present in Churg-Strauss | - Churg-Strauss is also associated with asthma, where as microscopic polyangiitis is not

Question 38

HSP

Answer 38

Henoch-Schonlein purpura (a small vessel vasculitis)

Question 39

HSP is the MOST common vasculitis in which demographic?

Answer 39

- (Henoch-Schonlein purpura) | - most common vasculitis in kids

Question 40

Patients with HSP commonly present with what symptoms?

Answer 40

- (Henoch-Schonlein purpura) | - palpable purpura on buttocks and legs

Question 41

HSP is due to IgA immune complex deposition in the blood vessel wall, explaining why it is usually seen following a ______________.

Answer 41

- (Henoch-Schonlein purpura) | - usually follows an upper respiratory tract infection