Vasculitides

Question 1

What are the vasculitides?

Answer 1

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

Question 2

Name the large vessel vasculitides

Answer 2

GCA
PMR
Takaysu’s Arteritis

Question 3

Name the medium vessel vasculitides

Answer 3

PAN
Kawasaki disease

Question 4

Name the small vessel vasculitides

Answer 4

WG
MPA
CSS
HSP
Cryoglobulinemic vasculitis
Hypersensitivity vasculitis

Question 5

Name the Antineurtrophil Cytoplasmic Antibody
(ANCA) Associated Vasculitides

Answer 5

WG
MPA
CSS

Question 6

Proximal muscle pain (NOT weakness)

Answer 6

Polymyalgia Rheumatica

Question 7

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

Answer 7

The shoulders and pelvic girdle muscles

Question 8

Polymyalgia Rheumatica is associated with which disease processes?

Answer 8

giant cell (temporal) arteritis
cancer

Question 9

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

Answer 9

bilateral and symmetrical

Question 10

What patient demographic is polymyalgia rheumatica commonly seen in?

Answer 10

older, white women

Question 11

Pneumonic for Polymyalgia Rheumatica: SECRET

Answer 11

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

Question 12

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

Answer 12

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

Question 13

Chronic vasculitis which affects large arterial branches of the aortic arch

Answer 13

Temporal (Giant Cell) Arteritis

Question 14

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

Answer 14

cranial vessels or aortic arch and branches

Question 15

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

Answer 15

Vision loss

Question 16

Definitive diagnosis of Temporal (giant cell) arteritis

Answer 16

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

Question 17

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

Answer 17

Temporal (giant cell) arteritis

Question 18

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

Answer 18

Polymyalgia Rheumatica

Question 19

“curtain coming down”

Answer 19

Amaurosis fugax

Question 20

“Pulseless disease”

Answer 20

Takayasu’s Arteritis

Question 21

Takayasu’s Arteritis targets what?

Answer 21

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

Question 22

What are the 6 criteria for Takayasu’s Arteritis?

Answer 22

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)

Question 23

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

Answer 23

Takayasu’s Arteritis

Question 24

What is the definitive diagnosis for Takayasu’s Arteritis?

Answer 24

angiography

Question 25

Which vasculitide is known as the classic vasculitis?

Answer 25

Polyarteritis Nodosa

Question 26

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

Answer 26

Polyarteritis Nodosa

Question 27

What organ does Polyarteritis Nodosa spare?

Answer 27

Lungs

Question 28

Which types of medication can cause Polyarteritis Nodosa?

Answer 28

Sulfa Gold penicillamine

Question 29

Which vasculitide is also known as Mucocutaneous lymph node syndrome?

Answer 29

Kawasaki Disease

Question 30

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

Answer 30

Kawasaki Disease

Question 31

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

Answer 31

Typically under 5 years of age

Question 32

What vessels does Kawasaki Disease primarily affect?

Answer 32

affects the coronary arteries

Question 33

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

Answer 33

Strawberry tongue (prominent papillae)

Question 34

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

Answer 34

ASA

Question 35

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

Answer 35

Necrotizing, granulomatous vasculitis

Question 36

What is Wegener’s Granulomatosis referred to as now?

Answer 36

Granulomatosis with Polyangiitis

Question 37

What is the Wegener’s Granulomatosis triad?

Answer 37

Upper respiratory symptoms Lower respiratory symptoms Focal segmental glomerulonephritis

Question 38

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

Answer 38

Wegener’s Granulomatosis

Question 39

How to diagnose Wegener’s Granulomatosis?

Answer 39

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

Question 40

c-ANCA is seen in which vasculitide?

Answer 40

Wegener’s Granulomatosis

Question 41

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

Answer 41

Microscopic Polyangiitis

Question 42

What are the five main clinical manifestations of Microscopic Polyangiitis?

Answer 42

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

Question 43

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%

Answer 43

Churg-Strauss Syndrome

Question 44

What vasculitide is also known as angiitis granulomatosis?

Answer 44

Churg-Strauss Syndrome

Question 45

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

Answer 45

the skin

Question 46

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

Answer 46

Cryobulinemia

Question 47

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

Answer 47

Hepatitis C (drug use)

Question 48

What is characteristic of Henoch-Schonlein Purpura?

Answer 48

IgA in vessel walls

Question 49

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

Answer 49

Henoch-Schonlein Purpura

Question 50

What are the two categories in Hypocomplementemic Urticarial Vasculitis Syndrome?

Answer 50

Hypocomplementemic Urticarial Vasculitis Hypocomplementemic Urticarial Vasculitis Syndrome (HUVS)

Question 51

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

Answer 51

Rule out SLE and cryoglobulinemia

Question 52

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

Answer 52

Behcet’s Disease

Question 53

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

Answer 53

HLA-B51

Question 54

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

Answer 54

Cogan’s Disease

Question 55

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

Answer 55

Cogan’s Disease

Question 56

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

Answer 56

Buerger’s Disease