Vasculitides

Question 1

Name the Large-Vessel Vasculitides

Answer 1

Giant Cell (Temporal) Arteritis
Takayasu Arteritis

Question 2

Name the Medium-Vessel Vasculitides

Answer 2

Polyarteritis Nodosa
Kawasaki Disease
Burger Disease (Thromboangiitis Obliterans)

Question 3

Name the Small-Vessel Vasculitides

Answer 3

Granulomatosis with Polyangiitis (Wegener)
Microscopic Polyangiitis
Eosinophilic Granulomatosis with polyangiitis (Churg-Strauss)
Henoch-Schonlein Purpura

Question 4

Medium-vessel vasculitis involves what type of arteries? HY

Answer 4

muscular arteries that supply organs

Question 5

Etiology of most vasculitis is what?

Answer 5

unknown and not infectious

Question 6

What arteries does Giant Cell Arteritis usually involve?

Answer 6

branches of the carotid artery

Question 7

what type of vasculitis is Giant Cell Arteritis?

Answer 7

Granulomatous (always with giant cell)

Question 8

What is the most common form of vasculitis in older adults?

Answer 8

Giant Cell Arteritis

Question 9

Giant Cell Arteritis usually effects what population?

Answer 9

usually affects older females

Question 10

What is polymyalgia rheumatica and what vasculitis is associated with it?

Answer 10

flu-like symptoms with joint and muscle pain

Giant Cell Arteritis

Question 11

What are the issues that arise from the arteries involved in giant cell arteritis?

Answer 11

headache - temporal
blindness - ophthalmic
jaw claudication

Question 12

What blood test is elevated in Giant Cell Arteritis?

Answer 12

ESR > 100

Question 13

Upon biopsy what do you see in giant cell arteritis?

Answer 13

inflamed vessel wall with giant cells and intimal fibrosis (focal granulomatous inflammation)

Question 14

How do you treat Giant Cell Arteritis?

Answer 14

corticosteroids

Question 15

Complication from not treating Giant Cell Arteritis? HY

Answer 15

high risk of blindness

Question 16

What type of vasculitis is Takayasu and what arteries does is classically involve?

Answer 16

granulomatous vasculitis classically affecting aortic arch at branch points

Question 17

Who gets Takayasu arteritis?

Answer 17

young Asian females

Question 18

What arteritis is known as pulseless disease and what does that present with?

Answer 18

Takayasu

weak or absent pulse in upper extremity

Question 19

What are some signs and symptoms of Takayasu?

Answer 19

flu-like symptoms with neurological and ocular disturbances along with a weak or absent upper extremity pulse

Question 20

What blood test is elevated in Takayasu?

Answer 20

ESR

Question 21

What is the treatment for Takayasu?

Answer 21

corticosteroids

Question 22

Polyarteritis Nodosa gets signs and symptoms in every organ except what?

Answer 22

lungs

Question 23

What presents in young adults as hypertension, abdominal pain with melena, neurologic disturbances, and skin lesions?

Answer 23

Polyarteritis Nodosa

Question 24

What serotype is positive in Polyarteritis Nodosa? (HY)

Answer 24

serum HBsAg (Hep B)

Question 25

Early vs Late lesions in Polyarteritis Nodosa?

Answer 25

early lesions consists of transmural inflammation with fibrinoid necrosis late lesions heals with fibrosis

Question 26

What vasculitis has strings-of-pearls appearance and what causes them? **HY**

Answer 26

Polyarteritis Nodosa? | Early lesions become aneurysms between the late fibrotic lesions

Question 27

What is the prognosis of Polyarteritis Nodosa?

Answer 27

fatal if left untreated

Question 28

What is the treatment for Polyarteritis Nodosa?

Answer 28

Corticosteroids and Cyclophosphamide

Question 29

What is the immune pathogenicity of Polyarteritis Nodosa?

Answer 29

immune complex mediated (Type III Hypersensitivity)

Question 30

Who gets Kawasaki Disease?

Answer 30

Asian children

Question 31

What is one way to remember who gets Kawasaki Disease?

Answer 31

think of a young asian child riding a kawasaki bike - palms and soles affected

Question 32

CRASH and Burn mnemonic for Kawasaki Disease?

Answer 32

``` Conjunctival injection Rash (polymorphous -> desquamating) Adenopathy (cervical) Strawberry tongue (oral mucositis) Hand-Foot erythematous rash fever ```

Question 33

Kawasaki Disease commonly can affect what arteries with what complications? **HY**

Answer 33

Coronary artery involvement leading to (1) thrombosis with MI and (2) aneurysm with rupture

Question 34

Treatment of Kawasaki Disease?

Answer 34

aspirin and IVIG

Question 35

Another name for Beurger Disease

Answer 35

Thromboangiitis Obliterans

Question 36

Who gets Thromboangiitis Obliterans (Beurger)?

Answer 36

heavy smokers, it is a smokers disease | Males

Question 37

What is often present in Thromboangiitis Obliterans (Beurger)? **HY**

Answer 37

Raynaud Phenomenon

Question 38

What type of vasculitis is Thromboangiitis Obliterans (Beurger)?

Answer 38

necrotizing vasculitis of digits

Question 39

How does Thromboangiitis Obliterans (Beurger) present?

Answer 39

gangrene, ulceration, and autoamputation of fingers and toes

Question 40

Treatment for Thromboangiitis Obliterans (Beurger)?

Answer 40

smoking cessation

Question 41

What is another name for Wegeners?

Answer 41

Granulomatosis with polyangiitis

Question 42

What type of vasculitis is Wegeners?

Answer 42

necrotizing granulomatous vasculitis

Question 43

Where does Wegeners commonly affect?

Answer 43

Nasopharynx, Lungs, and Kidneys

Question 44

What is the We"C"ener's mnemonic? **HY**

Answer 44

lots of C's with this disease C shape distribution of disease C-ANCA (PR3-ANCA) Cyclophosphamide and Corticosteroids treatment

Question 45

What vasculitis is classically seen in a middle aged male presenting with sinusitis, nasopharyngeal ulcers, hemoptysis, bilateral nodular lung infiltrates, and hematuria?

Answer 45

Granulomatosis with Polyangiitis (Wegeners)

Question 46

Why do Wegeners patients get hematuria? **HY**

Answer 46

rapidly progressive necrotizing glomerulonephritis

Question 47

What blood marker correlates with disease activity in Wegeners?

Answer 47

C-ANCA/PR3-ANCA

Question 48

What does biopsy in Wegeners show?

Answer 48

large necrotizing granulomas with adjacent necrotizing vasculitis

Question 49

What is the treatment for Wegeners? **HY**

Answer 49

Cyclophosphamide and corticosteroids

Question 50

Prognosis of Granulomatosis with polyangiitis?

Answer 50

relapses are common

Question 51

What is PR3-ANCA?

Answer 51

anti-proteinase-3

Question 52

What vasculitis is similar in presentation to Wegeners but lacks nasopharyngeal involvement and has no granulomas?

Answer 52

Microscopic Polyangiitis

Question 53

What kind of vasculitis is Microscopic Polyangiitis and what organ does it involve?

Answer 53

Necrotizing vasculitis involving especially lung, kidney and skin

Question 54

How does Microscopic Polyangiitis present?

Answer 54

pauci-immune glomerulonephritis and palpable purpura

Question 55

What blood marker correlates with disease activity in Microscopic Polyangiitis?

Answer 55

P-ANCA/MPO-ANCA

Question 56

How do you differentiate between Wegeners and Microscopic Polyangiitis?

Answer 56

Microscopic Polyangiitis has no nasopharyngeal involvement and no granulomas

Question 57

How do you treat Microscopic Polyangiitis?

Answer 57

corticosteroids and cyclophosphamide

Question 58

What kind of vasculitis is Churg-Strauss and what organs are involved?

Answer 58

Granulomatous, Necrotizing vasculitis with eosinophilia involving especially lungs and heart

Question 59

What vasculitis presents with asthma?

Answer 59

Churg-Strauss (Eosinophilic Granulomatosis with Polyangiitis)

Question 60

What vasculitis presents with peripheral neuropathy leading to wrist and foot drop?

Answer 60

Churg-Strauss (Eosinophilic Granulomatosis with Polyangiitis)

Question 61

What blood marker correlates with disease activity in Churg-Strauss?

Answer 61

P-ANCA/MPO-ANCA

Question 62

How do you differentiate between Microscopic Polyangiitis and Churg-Strauss?

Answer 62

both have MPO/P-ANCA Microscopic Polyangiitis has no asthma and no granulomas Churg Strauss has granulomas, eosinophilia, and asthma

Question 63

What type of kidney involvement is there in Churg-Strauss?

Answer 63

pauci-immune glomerulonephritis

Question 64

What does pauci-immune mean?

Answer 64

vasculitis that is associated with minimal evidence of hypersensitivity upon immunofluorescence.

Question 65

What is the most common vasculitis in children? **HY**

Answer 65

Henoch Schonlein Purpura

Question 66

What does Henoch Schonlein Purpura occurs following what?

Answer 66

upper respiratory tract infection

Question 67

What is Henoch Schonlein Purpura secondary to? **HY**

Answer 67

IgA immune complex deposition

Question 68

Pathogenesis of Henoch Schonlein Purpura **HY**

Answer 68

Upper respiratory infection leads to high IgA due to mucosal involvement --> accumulation of IgA --> IgA complex deposition --> vasculitis

Question 69

What is a major complication of igA deposition in Henoch Schonlein Purpura? **HY**

Answer 69

hematuria due to IgA nephropathy (Berger Disease)

Question 70

What sign occurs in Henoch Schonlein Purpura due to inflammation? **HY**

Answer 70

PALPABLE purpura on buttocks and legs

Question 71

Classic triad in Henoch Schonlein Purpura?

Answer 71

palpable purpura on buttocks/legs Arthralgias abdominal pain

Question 72

Treatment for Henoch Schonlein Purpura?

Answer 72

self limited disease but may recur, treat with steroids if severe

Question 73

What vasculitis presents with melena?

Answer 73

Polyarteritis Nodosa