Vasculitis Flashcards

1
Q

how do you divide vasculitis?

A

Large, medium and small

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2
Q

what are the types of large vessel vasculitis

A

Takayasu’s

Giant cell

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3
Q

What are the types of medium vessel vasculitis

A

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

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4
Q

Which medium vessel vasculitis is negative for blood markers?

A

Kawasaki’s
Polyarteritis nodosa
Behcet’s disease

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5
Q

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

A

Churg - Strauss (P-ANCA)

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6
Q

What are the types of small vessel vasculitis?

A
Henoch - Schonlein purpura
Essential cryoglobulinemia
arteritis of connective tissues disease (Scl, SLE, RA, MCTD)
Wegener's granulomatosis (C-ANCA)
Microscopic polyangiitis (P-ANCA)
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7
Q

which small vessel vasculitis are blood test negative?

A

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

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8
Q

which small vessel vasculitis are blood test positive?

A
Wegener's granulomatosis (C-ANCA)
Microscopic polyangiitis (P-ANCA)
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9
Q

Takayasu - Ueno Classification class 1?

A

localized to the arch of the aorta and branches

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10
Q

Takayasu - Ueno Classification class 2a?

A

ascending aorta, arch and branches

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11
Q

Takayasu - Ueno Classification class 2b?

A

ascending aorta, arch and branches, descending thoracic aorta

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12
Q

Takayasu - Ueno Classification class 3.

A

descending thoracic aorta, abdominal aorta +/- renal arteries

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13
Q

Takayasu - Ueno Classification class 4?

A

abdominal aorta +/- renal arteries

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14
Q

Takayasu - Ueno Classification class 5?

A

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

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15
Q

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

A

C- coronary

P - pulmonary

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16
Q

what’s Reynauld’s disease?

A

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

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17
Q

What’s Reynauld’s syndrome?

A

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

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18
Q

what’s the antibody for Scleroderma?

A

specled ANA

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19
Q

what’s the antibody for SLE?

A

homogenious ANA

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20
Q

what’s the antibody for CREST

A

Anticentromere Ab

21
Q

What’s the antibody for Sjogrens

A

SS Ab

22
Q

Diagnostic criteria for Giant cell Arteritis

A
  1. age >50
  2. localized headache
  3. temporal artery tenderness
  4. ESR >50
  5. temporal artery biopsy + for giant cell arteritis
23
Q

Diagnostic criteria for Behcet disease?

A
  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
24
Q

What are the aneurysms in Behcet disease?

A

aorta > pulmonary > femoral > popliteal > brachial > iliac

25
Q

Characteristics of Poliarteritis Nodosa?

A
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
26
Q

Characteristics of Kawasaki disease?

A
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

27
Q

Characteristics of Churg - STrauss?

A
  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
28
Q

Wegener’s disease characteristics?

A

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

29
Q

Differential diagnosis of digital ischemia

A
  1. ANCA + : microscopic polyangiitis and Wegener’s
  2. ANCA -: CTD, cryoglobulinemia
  3. Trauma: vibration, frost bite
  4. Embolic
30
Q

Types of fibromuscular displasia

A
  1. intimal fibroplasia
  2. medial fibroplasia
  3. perimedial fibroplasia
  4. adventitial fibroplasia/hyperplasia
31
Q

which type of FMD is most common

A

medial fibroplasia

32
Q

characteristics of intimal fibroplasia FMD

A

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

33
Q

characteristics of medial fibroplasia FMD

A

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

34
Q

characteristics of perimedial fibroplasia FMD

A

young girls and women, 5%

also string of beads but diameter of bead does not exceed proximal vessel

35
Q

characteristics of adventitial fibroplasia FMD

A

1:1 M:F

unifocal long stenosis

36
Q

Takayasu’s therapy

A

1mg/kg for 1 - 3 months

37
Q

medical therapy for Buerger’s

A
  1. antiplatelets
  2. calcium channel blockers
  3. prostaglanding analogues (Iloprost)
38
Q

segmental arterial mediolysis

A

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

39
Q

Moyamoya disease

A

“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)

40
Q

FMD in children

A

focal intimal fibroplasia

41
Q

Reynald’s disease duplex

A

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

42
Q

Williams syndrome

A

elfin features of face
supravalvular aortic stenosis
hypercalcemia
mild intellectual disability

43
Q

Loeys - Dietz syndrome

A
aortic aneurysms
vessel tortuosity
craniofacial abnormality
bifid uvula and cleft palate
hypertension
skeletal malformation 
food alergies
caused by mutation of TGF beta
44
Q

How much FMD in the neck is bilateral

A

65%

45
Q

For much FMD in the neck is associated with intracranial aneurysms

A

30%

46
Q

Diagnostic criteria for giant cell arteritis

A
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
47
Q

Surgical indications for Loeys - Dietz

A

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

48
Q

What’s the prophylaxis for Loeys Dietz syndrome

A

Angiotensin II receptor inhibitor

49
Q

Pedowitz criteria for chronic exertions compartment syndrome

A

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