Vasculitis

Question 1

how do you divide vasculitis?

Answer 1

Large, medium and small

Question 2

what are the types of large vessel vasculitis

Answer 2

Takayasu’s

Giant cell

Question 3

What are the types of medium vessel vasculitis

Answer 3

Kawasaki’s
Polyarteritis nodosa
Behcet’s disease
Churg - STrauss (P-ANCA positive)

Question 4

Which medium vessel vasculitis is negative for blood markers?

Answer 4

Kawasaki’s
Polyarteritis nodosa
Behcet’s disease

Question 5

Which medium vessel vasculitis is positive for blood marker and which marker?

Answer 5

Churg - Strauss (P-ANCA)

Question 6

What are the types of small vessel vasculitis?

Answer 6

Henoch - Schonlein purpura
Essential cryoglobulinemia
arteritis of connective tissues disease (Scl, SLE, RA, MCTD)
Wegener's granulomatosis (C-ANCA)
Microscopic polyangiitis (P-ANCA)

Question 7

which small vessel vasculitis are blood test negative?

Answer 7

Henoch - Schonlein purpura
Essential cryoglobulinemia
Arteritis of connective tissue disease (Scl, SLE, RA, MCTD)

Question 8

which small vessel vasculitis are blood test positive?

Answer 8

Wegener's granulomatosis (C-ANCA)
Microscopic polyangiitis (P-ANCA)

Question 9

Takayasu - Ueno Classification class 1?

Answer 9

localized to the arch of the aorta and branches

Question 10

Takayasu - Ueno Classification class 2a?

Answer 10

ascending aorta, arch and branches

Question 11

Takayasu - Ueno Classification class 2b?

Answer 11

ascending aorta, arch and branches, descending thoracic aorta

Question 12

Takayasu - Ueno Classification class 3.

Answer 12

descending thoracic aorta, abdominal aorta +/- renal arteries

Question 13

Takayasu - Ueno Classification class 4?

Answer 13

abdominal aorta +/- renal arteries

Question 14

Takayasu - Ueno Classification class 5?

Answer 14

ascending aorta, arch and branches, descending thoracic aorta and abdominal aorta +/- renal arteries

Question 15

What does C or P mean in Takayasu - Ueno classification?

Answer 15

C- coronary

P - pulmonary

Question 16

what’s Reynauld’s disease?

Answer 16

idiopathic, vasospastic, bilateral (Primary)
at room temperature patients have normal PVR
needs cold immersion test

Question 17

What’s Reynauld’s syndrome?

Answer 17

secondary to occlusive pathology or connective tissue disease
patients have abnormal PVRs at room temperature

Question 18

what’s the antibody for Scleroderma?

Answer 18

specled ANA

Question 19

what’s the antibody for SLE?

Answer 19

homogenious ANA

Question 20

what’s the antibody for CREST

Answer 20

Anticentromere Ab

Question 21

What’s the antibody for Sjogrens

Answer 21

SS Ab

Question 22

Diagnostic criteria for Giant cell Arteritis

Answer 22

1. age >50
2. localized headache
3. temporal artery tenderness
4. ESR >50
5. temporal artery biopsy + for giant cell arteritis

Question 23

Diagnostic criteria for Behcet disease?

Answer 23

1. major - oral ulcers
2. minor (needs two)
   a) genital ulcers
   b) eye lesions (uveitis 80%)
   c) skin - erythema nodosum
   d) venous thrombosis (50%)
   e) arterial pathology: aneurysms (AAA>pulmonary>femoral > popliteal>brachial>iliac) or occlusive disease
   f) other: pericarditis, arthritis, GI ulcers, CNS

Question 24

What are the aneurysms in Behcet disease?

Answer 24

aorta > pulmonary > femoral > popliteal > brachial > iliac

Question 25

Characteristics of Poliarteritis Nodosa?

Answer 25

``` systemic necrotizing vasculitis abdominal pain in young adults multiple mesenteric aneurysms increase ESR, normal ANCA 30% have positive hepatitis B, associated with HIV 2:1 male ```

Question 26

Characteristics of Kawasaki disease?

Answer 26

``` high fever, remittent for 2 weeks bilateral conjunctival inflammation strawberry tongue, swollen lips erythema of palmes and soles 20-30% coronary aneurysms ``` Coronary and brachiocephalic aneurysms in 25% of untreated patients MI and arrhythmia most frequent cause of pain Can be prevented with ASA and IV IG during acute phase Aneurysms <4mm universally egress and >8 mm don’t regress

Question 27

Characteristics of Churg - STrauss?

Answer 27

1. alergic phase - cold, asthma 2. eosinophilia with infiltrates (PNA, gastrienteritis, neuropathy) 3. vasculitis - most common coronary 4. P-ANCA positive 5. proximal vessel involvement

Question 28

Wegener's disease characteristics?

Answer 28

necrosing granulomatosus vasculitis kidney and upper respiratory tract lesion digital ischemia C-ANCA 90%

Question 29

Differential diagnosis of digital ischemia

Answer 29

1. ANCA + : microscopic polyangiitis and Wegener's 2. ANCA -: CTD, cryoglobulinemia 3. Trauma: vibration, frost bite 4. Embolic

Question 30

Types of fibromuscular displasia

Answer 30

1. intimal fibroplasia 2. medial fibroplasia 3. perimedial fibroplasia 4. adventitial fibroplasia/hyperplasia

Question 31

which type of FMD is most common

Answer 31

medial fibroplasia

Question 32

characteristics of intimal fibroplasia FMD

Answer 32

1:1 M:F, 10% young ringlike focal stenosis or long irregular tubular stenosis

Question 33

characteristics of medial fibroplasia FMD

Answer 33

adolescent, 80% 9:1 F:M string of beads, distal 2/3 of renal artery

Question 34

characteristics of perimedial fibroplasia FMD

Answer 34

young girls and women, 5% | also string of beads but diameter of bead does not exceed proximal vessel

Question 35

characteristics of adventitial fibroplasia FMD

Answer 35

1:1 M:F | unifocal long stenosis

Question 36

Takayasu's therapy

Answer 36

1mg/kg for 1 - 3 months

Question 37

medical therapy for Buerger's

Answer 37

1. antiplatelets 2. calcium channel blockers 3. prostaglanding analogues (Iloprost)

Question 38

segmental arterial mediolysis

Answer 38

simmilar to FMD but affects splanchnic vessels of middle aged and elderly individuals, can cause hypovolemic shock, PSA formation

Question 39

Moyamoya disease

Answer 39

"puff of smoke" cerebral proliferative angiopathy surgical revascularization for occluded carotid is beneficial in this condition (external carotid to internal carotid bypass or superficial temporal artery to MCA bypass)

Question 40

FMD in children

Answer 40

focal intimal fibroplasia

Question 41

Reynald's disease duplex

Answer 41

DPG wave with somewhat smaller upstroke with sharp anacrotic notch and dicrotic notch located unusually high

Question 42

Williams syndrome

Answer 42

elfin features of face supravalvular aortic stenosis hypercalcemia mild intellectual disability

Question 43

Loeys - Dietz syndrome

Answer 43

``` aortic aneurysms vessel tortuosity craniofacial abnormality bifid uvula and cleft palate hypertension skeletal malformation food alergies caused by mutation of TGF beta ```

Question 44

How much FMD in the neck is bilateral

Answer 44

65%

Question 45

For much FMD in the neck is associated with intracranial aneurysms

Answer 45

30%

Question 46

Diagnostic criteria for giant cell arteritis

Answer 46

``` At least theee of the following: 1. Age>50 2. Sudden onset of localized headache 3 temporal tenderness or decreased pulse 4. ESR >50 5. Histologic findings of inflammation ```

Question 47

Surgical indications for Loeys - Dietz

Answer 47

Aortic root >4 cm Descending thoracic aorta >5cm Infrarenal aorta >4cm

Question 48

What’s the prophylaxis for Loeys Dietz syndrome

Answer 48

Angiotensin II receptor inhibitor

Question 49

Pedowitz criteria for chronic exertions compartment syndrome

Answer 49

Normal resting compartment pressure of >15mmHg >30mmHg 1 min post exercise >20mmHg 5 min post exercise