7.1 Vasculitis

Question 1

What is vasculitis?

Answer 1

Inflammation of blood vessel wall

Question 2

What are the symptoms of vasculitis?

Answer 2

• Nonspecific

- Organ ischemia

Question 3

What does large vessel vasculitis tend to affect?

Answer 3

Aorta and its branches

Question 4

What are the types of large vessel vasculitides?

Answer 4

• Giant Cell Arteritis (GCA)

- Takayasu’s Arteritis

Question 5

What is the typical patient of GCA?

Answer 5

Older females (>50)

Tends to affect Caucasians

Question 6

What is the classic location of GCA?

Answer 6

Branches of the carotid

• temporal artery
• ophthalmic artery causing visual disturbance

Question 7

What is seen on LM of GCA?

Answer 7

Granulomatous vasculitis with giant cells and intimal fibrosis

Question 8

Why must a long piece be taken for GCA biopsy?

Answer 8

The lesions are segmental - negative biopsies may not exclude the disease

Question 9

How does GCA cause ischemia?

Answer 9

Fibrosis in between the intimal and the media that leads to narrowing of the lumen which decreases perfusion

Question 10

What is the treatment for GCA?

Answer 10

Corticosteroids - treat ASAP

Question 11

What is the typical patient of Takayasu’s Arteritis?

Answer 11

Adults less than the age of 50 - typically an Asian female

Question 12

What does LM of Takayasu’s Arteritis show?

Answer 12

Granulomatous vasculitis

Question 13

What vessels does Takayasu’s Arteritis tend to affect?

Answer 13

Aortic arch at the branch points

Question 14

What are some clinical signs of Takayasu’s Arteritis?

Answer 14

• Weak or absent pulse in the upper extremities

- ESR is elevated

Question 15

What is the treatment for Takayasu’s Arteritis?

Answer 15

Corticosteroids

Question 16

What does medium vessel vasculitis tend to affect?

Answer 16

Muscular arteries that supply organs

Question 17

What are the types of medium vessel vasculitis?

Answer 17

• Polyarteritis Nodosa (PAN)
• Kawasaki Disease
• Buerger Disease

Question 18

What are the effects of polyarteritis nodosa?

Answer 18

Necrotizing vasculitis that involves multiple organs

Question 19

What organ is spared by PAN?

Answer 19

Lungs

Question 20

What is the characteristic finding of PAN on imaging?

Answer 20

“String on pearls” - aneurysms between fibrous areas of the vessel

Question 21

What are some of the characteristics of PAN?

Answer 21

• Fibrinoid necrosis

- Varying stages of disease along the same artery

Question 22

What is the the treatment for PAN?

Answer 22

Corticosteroids
Cyclophosphamide
-Fatal if not treated

Question 23

What is the typical patient of Kawasaki Disease?

Answer 23

Asian children less than the age of 4

Question 24

What are the presentations of Kawasaki Disease?

Answer 24

Fever
Conjunctivitis
Erythematous rash of palms and soles - “motorcycle”

Question 25

What is the preferential artery involved in Kawasaki Disease?

Answer 25

Coronary Artery

Question 26

What are the complications of Kawasaki Disease?

Answer 26

- MI | - Aneurysm and rupture

Question 27

What is the treatment of Kawasaki Disease and why is it unique?

Answer 27

Aspirin and IVIG | - Normally you would NEVER give a child aspirin as it risks Reye's Disease

Question 28

How does aspirin treat Kawasaki Disease?

Answer 28

Aspirin prevents TXA2 to prevent thrombus formation in the coronary artery

Question 29

What is Buerger Disease?

Answer 29

Necrotizing vasculitis of the digits that presents with ulcers and gangrene

Question 30

What is Buerger Disease associated with?

Answer 30

Smoking

Question 31

What is the treatment for Buerger Disease?

Answer 31

Cessation of smoking

Question 32

What is a sign of Buerger Disease?

Answer 32

Raynaud's Phenomenon

Question 33

What does small vessel vasculitis affect?

Answer 33

Arterioles, capillaries and venules

Question 34

What is Wegener's Granulomatosis?

Answer 34

Necrotizing granulomatous vasculitis that involves the nasopharynx, lungs and kidneys

Question 35

What is the pathogenesis of Wegener's Granulomatosis?

Answer 35

c-ANCA

Question 36

What is the typical patient of Wegener's Granulomatosis?

Answer 36

Middle aged male with sinusitis or nasopharyngeal ulceration and hemoptysis with hematuria

Question 37

What is the treatment for Wegener's Granulomatosis?

Answer 37

Cyclophosphamide | Corticosteroids

Question 38

What is Microscopic Polyangiitis?

Answer 38

Necrotizing vasculitis with lung and kidney involvement

Question 39

What are the difference between Microscopic Polyangiitis and Wegener's?

Answer 39

- No nasopharyngeal - No granulomas - pANCA instead of cANCA

Question 40

What is the treatment for Microscopic Polyangiitis?

Answer 40

Cyclophosphamide | Corticosteroids

Question 41

What is Churg-Strauss Syndrome?

Answer 41

Necrotizing granulomatous vasculitis with eosinophils

Question 42

What is the pathogenesis of Churg-Strauss Syndrome?

Answer 42

pANCA

Question 43

What are the differences between Churg-Strauss Syndrome and Microscopic Polyangiits?

Answer 43

CS wil have: - Granulomas - Eosinophilia - Asthma

Question 44

What is the most common vasculitis in children?

Answer 44

Henoch-Shonlein Purpura

Question 45

What is the pathogenesis of Henoch-Shonlein Purpura?

Answer 45

Deposition of IgA following an URI due to excessive production

Question 46

What are the signs of Henoch-Shonlein Purpura?

Answer 46

- Palpable purpura on buttocks and legs | - Hematuria with IgA nephropathy

Question 47

What does Henoch-Shonlein Purpura tend to follow?

Answer 47

Respiratory tract infection

Question 48

What is the treatment for Henoch-Shonlein Purpura?

Answer 48

Self-limiting - use steroids only in severe cases