Vasculitides Flashcards

1
Q

What are the vasculitides?

A

A group of diseases characterized by non-infectious necrotizing vasculitis and resultant ischemia

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2
Q

Name the large vessel vasculitides

A

GCA
PMR
Takaysu’s Arteritis

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3
Q

Name the medium vessel vasculitides

A

PAN
Kawasaki disease

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4
Q

Name the small vessel vasculitides

A

WG
MPA
CSS
HSP
Cryoglobulinemic vasculitis
Hypersensitivity vasculitis

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5
Q

Name the Antineurtrophil Cytoplasmic Antibody
(ANCA) Associated Vasculitides

A

WG
MPA
CSS

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6
Q

Proximal muscle pain (NOT weakness)

A

Polymyalgia Rheumatica

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7
Q

Polymyalgia Rhematica is characterized by diffuse pain and stiffness primarily affecting what part(s) of the body?

A

The shoulders and pelvic girdle muscles

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8
Q

Polymyalgia Rheumatica is associated with which disease processes?

A

giant cell (temporal) arteritis
cancer

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9
Q

Is Polymyalgia rheumatica:
unilateral or bilateral?
symmetrical or asymmetrical?

A

bilateral and symmetrical

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10
Q

What patient demographic is polymyalgia rheumatica commonly seen in?

A

older, white women

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11
Q

Pneumonic for Polymyalgia Rheumatica: SECRET

A

S – symmetric proximal stiffness/pain
E – elderly
C – constitutional symptoms/Caucasian
R – rheumatism (arthritis)
E – ESR elevated
T – temporal arteritis

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12
Q

In a patient with polymyalgia rheumatica, when should you suspect cancer?

A

Suspect when ESR fails to normalize (Cancer may present as PMR)

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13
Q

Chronic vasculitis which affects large arterial branches of the aortic arch

A

Temporal (Giant Cell) Arteritis

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14
Q

Temporal (giant cell) arteritis most commonly involve which vessels?

A

cranial vessels or aortic arch and branches

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15
Q

What is the most ominous sign in temporal (giant cell) arteritis?

A

Vision loss

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16
Q

Definitive diagnosis of Temporal (giant cell) arteritis

A

biopsy (multiple)
Caution: skip lesions with this disease so may need multiple biopsy locations

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17
Q

Which disease should you expect if you see tender inflamed, cordlike temporal arteries with absent pulse on physical exam?

A

Temporal (giant cell) arteritis

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18
Q

What disease process is constantly seen with temporal (giant cell) arteritis?

A

Polymyalgia Rheumatica

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19
Q

“curtain coming down”

A

Amaurosis fugax

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20
Q

“Pulseless disease”

A

Takayasu’s Arteritis

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21
Q

Takayasu’s Arteritis targets what?

A

Targets aorta and major branches (Subclavian > aortic arch > ascending aorta > carotids > femoral)
Also known as “aortic arch syndrome”

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22
Q

What are the 6 criteria for Takayasu’s Arteritis?

A

Age of disease onset ≤ 40 years
Claudication of upper extremities
Decreased brachial artery pulse
Blood pressure difference >10 mm Hg between arms
Bruit over subclavian arteries or aorta
Arteriogram abnormality (Narrowing of the aorta or a major branch)

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23
Q

Which of the following vasculitides is described below?
Chronic vasculitis, unknown etiology
80-90% women
Age of onset 10-40
Most cases from Asia and Africa
Asian women <40 year old most common

A

Takayasu’s Arteritis

24
Q

What is the definitive diagnosis for Takayasu’s Arteritis?

A

angiography

25
Q

Which vasculitide is known as the classic vasculitis?

A

Polyarteritis Nodosa

26
Q

Which vasculitide results from Hepatitis B 30% of the time?

A

Polyarteritis Nodosa

27
Q

What organ does Polyarteritis Nodosa spare?

A

Lungs

28
Q

Which types of medication can cause Polyarteritis Nodosa?

A

Sulfa

Gold

penicillamine

29
Q

Which vasculitide is also known as Mucocutaneous lymph node syndrome?

A

Kawasaki Disease

30
Q

Which of the vasculitides is primarily in children, known as a childhood vasculitis?

A

Kawasaki Disease

31
Q

What is the typical age range for kids diagnosed with Kawasaki Disease?

A

Typically under 5 years of age

32
Q

What vessels does Kawasaki Disease primarily affect?

A

affects the coronary arteries

33
Q

What physical exam finding should alert you immediately to Kawasaki disease?

A

Strawberry tongue (prominent papillae)

34
Q

What is the initial treatment for Kawasaki Disease that is typically CI in children?

A

ASA

35
Q

What is the hallmark pathologic lesion for Wegener’s Granulomatosis?

A

Necrotizing, granulomatous vasculitis

36
Q

What is Wegener’s Granulomatosis referred to as now?

A

Granulomatosis with Polyangiitis

37
Q

What is the Wegener’s Granulomatosis triad?

A

Upper respiratory symptoms
Lower respiratory symptoms
Focal segmental glomerulonephritis

38
Q

What vasculitide is described in the following description:
Can be fatal – even if you treat it
“antibody to neutrophils in their own cytoplasm”
Caucasians most common

A

Wegener’s Granulomatosis

39
Q

How to diagnose Wegener’s Granulomatosis?

A

Labs: c-ANCA (Antineutrophil cytoplasmic antibody)
Definitive: biopsy

40
Q

c-ANCA is seen in which vasculitide?

A

Wegener’s Granulomatosis

41
Q

Which of the following vasculitides is described below:
Affects everybody
Present with palpable purpura
Can test with ANCA (Will be ANCA positive 70%)

A

Microscopic Polyangiitis

42
Q

What are the five main clinical manifestations of Microscopic Polyangiitis?

A

Kidney inflammation ~80% (Segmental necrotizing glomerulonephritis)
Weight loss >70%
Skin lesions >60%
Nerve damage >60% (Vasculitis neuropathy)
Fevers >55%

43
Q

Which vasculitide is described below:
Consists of small vessel vasculitis, extra vascular granulomas, and
hypereosinophilia
In patients with pre-existing asthma and allergic rhinitis
ANCA positive 50%

A

Churg-Strauss Syndrome

44
Q

What vasculitide is also known as angiitis granulomatosis?

A

Churg-Strauss Syndrome

45
Q

What organ of the body is always involved in Hypersensitivity Vasculitis?

A

the skin

46
Q

Which vasculitide is known as the “cold antibody in the blood”?

A

Cryobulinemia

47
Q

In Cryobulinemia, 90% of cases are associated with what?

A

Hepatitis C (drug use)

48
Q

What is characteristic of Henoch-Schonlein Purpura?

A

IgA in vessel walls

49
Q

What vasculitide is described below:
Immune complex-mediated vasculitis
Small vessel vasculitis
Occurs after URI or drugs
IgA in vessel walls
Males > females

A

Henoch-Schonlein Purpura

50
Q

What are the two categories in Hypocomplementemic Urticarial
Vasculitis Syndrome?

A

Hypocomplementemic Urticarial Vasculitis
Hypocomplementemic Urticarial Vasculitis Syndrome (HUVS)

51
Q

In Hypocomplementemic Urticarial Vasculitis, what do you need to rule out?

A

Rule out SLE and cryoglobulinemia

52
Q

Vasculitis with triad of recurrent oral and genital ulcers, and uveitis or
iritis

A

Behcet’s Disease

53
Q

What is a strong risk factor for Behcet’s Disease?

A

HLA-B51

54
Q

Which vasculitide is described below:
Rare disease of young adults
Vestibuloauditory dysfunction, interstitial keratitis, and non-reactive
syphilis test
Follows URI
Questionable association with chlamydia

A

Cogan’s Disease

55
Q

What vasculitide looks just like Meniere’s
disease – except for the age

A

Cogan’s Disease

56
Q

Thromboangiitis obliterans
Acute inflammation, thrombosis of arteries and veins of hands and
feet

A

Buerger’s Disease