Vasculitis

Question 1

How do immune processes leading to vasculitis progress in the large vessel?

Answer 1

outside-in

1. Activation of naive t-cells by tissue resident DCs in adventitia
2. oxidative damage
3. Non-stenosing arteritis (window of opportunity)
4. hypertrophy of intima and lumen occlusion. symptoms occur. scar is forming
5. On top of chronic stenosis you can get a thrombosis and the intima gets thicker–>occlusion
6. breakdown of internal elastic lamina–>aneurysm formation

Question 2

How do immune processes leading to vasculitis progress in the small/medium vessel?

Answer 2

inside out

a. Immune complexes
b. Antineutrophil antibodies
c. direct endothelial adhesion
- primary neutrophil
- neutrophil goes in and degranulates and causes inflammation

small–> breaking open of the artery

Question 3

What are common features of all vasculitides?

Answer 3

1. fatigue
2. fever
3. muscle pain
4. weight loss

• *CRP
• *Fibrinogen (sedimentation rate)

Question 4

What is the pathophysiology of small vessels?

Answer 4

1. Fibrinoid Necrosis
   - fibrin deposition –>breaking open the blood vessel
   - dead neutrophils fibrin deposition–>fibrin necrosis

Question 5

What are typical findings in small vessel vasculitis?

Answer 5

1. palpable purpura
2. necrotizing glomerulonephritis
3. pulmonary capillaritis and alveolar hemorrhage

Question 6

What is the pathophysiology of medium vessels?

Answer 6

1. focal lesions with fibrinoid necrosis
2. segmental lesions
3. aneurysms

Question 7

What are typical clinical findings of medium vessel vasculitis?

Answer 7

1. skin nodules
2. livedo racemosa
3. ulceration
4. nerve infarcts
5. abdominal pain

Question 8

What is the pathophysiology of large vessels?

Answer 8

1. cell mediated
   - not neutrophil or immune complex
2. granulomas usually present
3. Hyperplastic intima with stenosis
   - with neovascularization
4. FIBRINOID NECROSIS UNUSUAL

Question 9

What are typical clinical findings of large vessel vasculitis?

Answer 9

1. ocular symptoms
2. Claudication
3. chest pain
4. headache stroke

Question 10

What type of study do you use to look at large and medium arteries?

Answer 10

Angiography or MRA

Question 11

What type of study do you use to look at small and medium arteries?

Answer 11

1. CT chest
2. lung/kidney biopsy
3. skin biopsy

Question 12

C-ANCA

Answer 12

PR3

Question 13

P-ANCA

Answer 13

MPO

Question 14

What are 4 clinical diagnosis considerations?

Answer 14

1) vessel size
2) anca association
3) presence of granulomas
4) possible disease association

Question 15

What vasculitides have granulomas?

Answer 15

1. Giant cell arteritis
2. takayasu arteritis
3. wegener granulomatosis
4. churg strauss

Question 16

Giant Cell Arteritis
Epidemiology
Vessel

Answer 16

Epidemiology:
1. greater than 50, white, female

2. a. Carotid
   i. External
   - especially temporal arteries
   ii. Internal
   - ophthalmic
   - not intracerebral

b. Upper aortic branches
i. vertebral arteries

NO STRoKES

Question 17

What are the pathological features of GCA?

Answer 17

1. patchy/segmental
2. granulomatous inflammation
   - focused on the internal elastic membrane
3. fragmentation of internal elastic membrane

chronic-may be smouldering even if clinically quiet

Question 18

What are clinical features of GCA?

Answer 18

1. headaches
2. polymyalgia rheumatica
3. scalp tenderness **
4. TA (temporal artery) pulse abnormality **
5. ocular disturbances
6. visual loss
7. extreme claudication
8. cardiac or neurologic symptoms

Question 19

How do you investigate for GCA?

Answer 19

temporal artery biopsy**

blood vessels imagin

Question 20

What is the treatment for GCA?

Answer 20

corticosteroids with slow taper

Question 21

What is the epidemiology and artery for pulseless disease (takayasu’s arteritis)?

Answer 21

50 or less
asian female

Aorta Great
Great vessels

STROKES
-favors bifurcation areas

Question 22

What are the pathologic features of takayasu’s?

Answer 22

1. giant cells (more spread)
2. transmural scarring more intense
3. severe hyperplastic intima with neovascularization
   - severe luminal narrowing

Question 23

What are the three phases of takayasu’s arteritis?

Answer 23

1. pre-pulseless, inflammation period. patients under 20 usually present in this phase
2. vessel inflammation dominated by vessel pain and tenderness
3. fibrotic stage, when bruits and ischemia predominate
   - arterial bypass or angioplasty for things like heart failure and htn often needed*

Question 24

What are the clinical features of takayasu’s?

Answer 24

1. abnormal pulses
2. claudication/weakness
3. pulmonary hypertension
4. carotidynia

Question 25

What is fundamental to diagnosis of takayasu's?

Answer 25

Blood vessel imaging** Echocardiography - non specific lab tests - ESR can be normal in 50%

Question 26

What is the epidemiology of kawasaki's?

Answer 26

children

Question 27

What are the pathological features of kawaskis?

Answer 27

self limiting | -anti-endothelial/smooth muscle cell antibodies

Question 28

What is window of opportunity to treat in kawasaki?

Answer 28

Stage 1: 0-2 weeks after fever -perivasculitis of microvessels Other stages Stage 2: 2-4 weeks -progression to medium vessel vasculitis -thrombosis and aneurysms Stage 3: reduction in inflammation Stage 4: scar with weakened wall and intimal thickening

Question 29

What are clinical features of kawasaki's?

Answer 29

fever rash bilateral conjunctivitis, strawberry tongue, extremity changes, cervical lymphadenopathy, MI

Question 30

What do you do to treat kawasaki's?

Answer 30

IVIG High dose aspirin within 1st 10 days of fever incidence of coronary aneurysm 25-->3-5%

Question 31

What is the epidemiology of PAN?

Answer 31

medium vessel uncommon, all ages associated- autoimmune heme malignancies viruses- HEP B 30% *****

Question 32

What vessels are affected by PAN?

Answer 32

medium to small MUSCULAR arteries - renal - coronary - hepatic

Question 33

What are the pathologic features of PAN?

Answer 33

1. focal * 2. segmental * 3. transmural destruction * 4. coexisting stages of activity* 5. fibrinoid necrosis 6. pleomorphic cellular infiltrate 7. thrombosis 8. aneurysms

Question 34

What are clinical features of PAN?

Answer 34

1. organ infarcts * 2. abdominal injury 3. renal injury/ hypertension 4. cutaneous lesions 5. arthralgia/arthritis 6. acute peripheral neuropathy - pain/paresthesia - motor deficits hours later - mononeuritis multiplex

Question 35

Does PAN need treatment? What is the treatment?

Answer 35

rapidly progressive and fatal if not treated Treatment -corticosteroids -HBV associated-plasma exchange, antiviral treatment -cyclophosphamide chemotherapy if severe

Question 36

Out of GPA (granulomatosis with polyangiitis-Wegners) , MPA (microscopic polyangiitis) and CS (churg-strauss) which are associated with granulomas?

Answer 36

Granulomatosis with polyangiitis Churg strauss

Question 37

What clinical feature of granulomatosis with polyangiitis?

Answer 37

- Destructive upper airway disease - pulmonary nodules/cavities/infiltrates - Necrotizing crescentic glomerulonephritis 1. destructive sinusitis 2. subglottis stenosis 3. gingival hyperplasia 4. orbital pseudotumor 5. necrotizing scleritis C-ANCA/PR3

Question 38

What clinical feature of microscopic polyangiitis?

Answer 38

Alveolar hemorrhage -Necrotizing crescentic glomerulonephritis P-ANCA/MPO

Question 39

What clinical feature of churg strauss?

Answer 39

Nasal polyps/allergic rhitnitis Asthma, allergy, eosinophilia -Necrotizing crescentic glomerulonephritis -cardiomyopathy due to eosinophilic cardiac infiltrate P-ANCA/MPO

Question 40

What do you see in the urine of these three small vessel things?

Answer 40

proteinuria | RBC casts +/-

Question 41

What is epidemiology of HSP?

Answer 41

most frequently children -can occur in adults small vessels -arterioles, capillary, or venules

Question 42

What are the pathologic features of HSP?

Answer 42

immune complex deposition | IgA and C3

Question 43

What are the clinical features of HSP?

Answer 43

palpable purpura arthritis abdominal pain renal impairment

Question 44

Buerger?

Answer 44

smoking disease medium and small arteries gangrene of extremities corkscrew collaterals

Question 45

Which diseases can be treated with steroids?

Answer 45

1. GCA 2. Takayasu's 3. HSP