Vasculitis

Question 1

Define vasculitis

Answer 1

Presence of leukocytes in the vessel wall WITH reactive damage to mural structures. This leads to tissue damage and necrosis

Question 2

Why does Vasculitis occur?

Answer 2

Occurs as a primary process or may be secondary to another underlying disease - exact mechanisms underlying these disorders are unclear.

Question 3

What are some pathogenic models of vasculitis? (5)

Answer 3

1. Antigen distribution
2. Role of Endothelial cell
3. Nonendothelial structures of vessel wall
4. Infectious agents (either by direct or indrect[immune complexes] vessel wall involvement
5. Drugs (cocaine)

Question 4

Theory for how endothelial cells are involved in vasculitis

Answer 4

• expression of adhesion molecules
• secretion of peptides and hormones
• interaction with inflammatory cells
• some endothelial cells are able to attract inflammatory cells while others are not
  
  • accounts for the sites of certain vasculopathies

Question 5

List three types of vasculitis affecting medium-vessels

Answer 5

`1. Polyarteritis nodosa

2. Kawasaki disease
3. Buerger’s disease (Thromboangiitis obliterans)

Question 6

What is Giant Cell arteritis?

Answer 6

Granulomatous vasculitis, vessel inflammation prominently involves the cranial branches of the arteries originating from the aortic arch:

• temporal, ophthalmic, posterior ciliary arteries, subclinical involvement of the aorta

Question 7

Define Takayasu’s arteritis

Answer 7

Granulomatous thickenin gof aortic arch, proximal great vessels. Affects the aorta and its branches (also pulmonary arteries), most commonly affects young women and children, particularly asians.

Question 8

“Pulseless disease” - what is it, what disease presents like this?

Answer 8

Weak upper extremity pulses, fever, night sweats, arthritis, myalgias, skin nodules, ocular disturbances.

Takayasu’s arteritis

Question 9

pathology/labs of Gian Cell arteritis

Answer 9

Most commonly affects branches of carotid artery; focal granulomatous inflammation, increased ESR and CRP

Question 10

Epidemiology/presentation of Giant Cell Arteritis

Answer 10

• generally elderly females
• unilateral HA, (temporal artery), jaw claudication (pain caused by too little blood flow)
• may lead to irreverible blindness due to ophthalmic artery occlusion
• associated with polymyalgia rheumatica
• scalp tenderness/skin necrosis (involving area where temporal artery is)

Question 11

What vessels does Polyarteritis Nodosa affect?

Answer 11

typically involves renal and visceral vessels, NOT pulmonary arteries

Question 12

Peak age onset of Polyarteritis Nodosa. What is a common association?

Answer 12

40-60 years

associated with HBV (50%)–usually recent infection

Question 13

2 characteristic findings of Polyarteritis Nodosa

Answer 13

1. Aneurysms
2. Stenosis

Question 14

Anti-neutrophil cytoplasmic antibody (ANCA) is asspcoated with 3 major vasculitities:

Answer 14

1. Wegener’s granulomatosis *c-ANCA*cytoplasmic
2. Microscopic Polyangiitis *p-ANCA*perniculear
3. Churg-Strauss syndrome *p-ANCA*perinuclear

Question 15

List 6 important small vessel vasculitis and common associations

Answer 15

1. Wegener’s granulomatosis : c-ANCA
2. Churg-Strauss Arteritis: p-ANCA (40%)
3. Microscopic Polyarteritis: p-ANCA
4. Henoch-Scholein purpura : IgA nephopathy
5. Essential Cryoglobulinemic Vasculitis
6. Cutaneous leukocytoclastic angiitis

Question 16

what happens when you palpate red nodular areas in palpable purpura that presents in Churg-Strauss syndrome?

Answer 16

they don’t blanch. Vasculitis manifestations don’t blanch (turn white)

Question 17

WHat is the manamgenet of vasculitis depend on?

Answer 17

therapy depends on the nature and severity of the vasculitis (withdrawal of the offending agent=drug, treatment ofunderlying infection

Question 18

2 options for systemic vasculitis

Answer 18

1. Corticosteroids is mainstay therapy (quickly: prednisone!!)
2. Cytotoxic agents: Cyclophosphamide, Methotrexate, Azothiaprine/Cyclosporin, Rituxamab)