Vasculitis

Question 1

The two general ways that vasculitis can lead to an ischemic state to an organ are:

Answer 1

exposure of subendothelial collagen (thrombus)

inflammation/fibrosis = stenosis

Question 2

The two vasculitis disorders to know that involve LARGE vessels are:

Answer 2

Temporal/giant cell arteritis

Takayasu arteritis

Question 3

Giant Cell Arteritis is to ELDERLY females as Takayasu arteritis is to ___________

Answer 3

Asian females < 40

Question 4

Takayasu’s Arteritis involves the ___________, while Giant Cell Arteritis involves the ___________

Answer 4

aortic arch

branches of carotid

Question 5

Which large vessel arteritis will have an elevated ESR (>100) and show granulomatous inflammation?

Answer 5

BOTH Giant Cell and Takayasu!

Question 6

Given that Giant Cell Arteritis involves the carotid artery branches, describe the patient’s symptoms according to what artery is involved.

Answer 6

Unilateral headache (temporal a)
Jaw claudication
Blindness (ophthalmic a)

Question 7

Which large vessel vasculitis can be associated with polymyalgia rheumatica?

Answer 7

Giant cell arteritis

GCA+fever, malaise, myalgia, arthralgia

Question 8

How do we differentiate between Giant Cell Arteritis and Optic Neuritis associated with MS?

Answer 8

similar patients!

Giant Cell: painless blindness
MS: painful blindness

Question 9

Pt presents with weak upper extremity pulses. She’s got an elevated ESR. Is she elderly or young?

Answer 9

most likely young (Asian)

describes ‘pulseless disease’; aka Takayasu arteritis

Question 10

If a pt with suspected Giant Cell arteritis comes in with a negative punch biopsy of the temporal artery, what the hell do ya think?

Answer 10

REDO the bx! (take a long ass strip of artery)

Giant Cell arteritis is FOCAL (can skip areas)

Question 11

The three Medium vessel vasculitis disorders to know are?

Answer 11

Polyarteritis nodosa
Kawasaki disease
Buerger’s disease (thromboangiitis obliterans)

Question 12

Contrast the classic patients characteristics for the 3 medium vessel vasculitis diseases.

Answer 12

Polyarteritis nodosa: young adult, maybe IVDU
Kawasaki disease: Asian kid < 4
Buerger’s disease: Heavy smoker

Question 13

_________ is a medium vessel vasculitis that can involve any artery EXCEPT the pulmonary vessels

Answer 13

Polyarteritis nodosa

Question 14

__________ is a medium vessel vasculitis that involves the digits

Answer 14

Buerger’s disease

Question 15

__________ is a medium vessel vasculitis involving the Coronary artery

Answer 15

Kawasaki disease

Question 16

A 3 year old Asian boy presents with a STEMI. What would have been a good treatment to prevent his heart attack?

Answer 16

IVIG + ASA (say whaaaaaaat)

Question 17

Giving ASA to a kid can lead to _______, but its good for Kawasaki disease to do what?

Answer 17

Reye’s syndrome

stop clot formation

Question 18

How do patients with Kawasaki disease present?

Answer 18

Non-specfic symptoms, sounds infectious!

fever, LAD, conjunctivitis, strawberry tongue, hand/foot rash, desquamation

Question 19

What does the name polyarteritis nodosa tell us about the findings?

Answer 19

many arteries (except lung)

nodosa = string of pearls on angiogram (lesions of different ages; aneurysms between fibrinoid necrosis)

Question 20

Polyarteritis nodosa is a type ______ hypersensitivity, and is often associated with seropositivity for _____________

Answer 20

type III HS

seropositive for Hep B (HBsAg)

Question 21

Pt presents with melena, ab pain, and HTN. He’s 20 and is + for HBsAg. What is the best method of tx?

Answer 21

Corticosteroids or Cyclophosphamide

tx for Polyarteritis nodosa

Question 22

Pt presents with claudication and multiple ulcers on his fingers. I’ve got a hunch that he has a long hx of _______ and his fingers are at risk for ________

Answer 22

smoking

autoamputation

Question 23

What autoantibody do we always want to think about with Small vessel vasculitis?

Answer 23

ANCA (anti-neutrophilic antibody)

Question 24

Compare the ANCA positivities in small vessel vasculitides.

Answer 24

Microscopic polyangiitis: p-ANCA
Churg-Strauss syndrome: p-ANCA
Wegener granulomatosis: c-ANCA

Question 25

What are the key differences between microscopic polyangiitis and Churg-Strauss syndrome?

Answer 25

MP: NO granulomas + NO asthma + NO Eosinophilia + lung/kidney CSS: Granulomas + asthma + Eosinophilia + lung/heart

Question 26

What are the key 3 areas involved with Wegener granulomatosis?

Answer 26

URT LRT Renal

Question 27

What are the key differences between microscopic polyangiitis and Wegener granulomatosis?

Answer 27

MP: NO granulomas + p-ANCA + NO URT involvement WG: Granulomas + c-ANCA + URT involvement

Question 28

What is the tx for the 3 ANCA small vessel vasculitides

Answer 28

Cyclophosphamide or Corticosteroids

Question 29

Henoch-Schonlein purpura is a small vessel vasculitis that classically follows a ________

Answer 29

upper respiratory tract infection in kids

Question 30

HSP classically presents as what 4 things?

Answer 30

PALPABLE purpura on glutes/hammies Arthralgia GI issues (pain, melena) Renal deposits

Question 31

What characteristic lab is found in HSP?

Answer 31

hi serum IgA (complexes cause dz)

Question 32

Compare + Contrast the kidney involvement of Wegener/microscopic polyangiitis with HSP

Answer 32

Both: Nephritic (RBC casts, hematuria, azotemia, oliguria, lo proteinuria) W: rapidly progressive (RPGN); CRESCENT-MOON shaped glomeruli (due to fibrin, C3b, et al) HSP: Berger's dz (IgA deposition in mesangium)

Question 33

Pt presents with hematuria and RBC casts in urine. Glomeruli show crescentic pattern. It is also found that there is inflammation of the small vessels of the lungs. What is the next best test to run?

Answer 33

c-ANCA (Wegener) vs p-ANCA (microscopic polyangiitis)

Question 34

Sturge-Weber syndrome (aka ___________) involves ________ vessels that typically follow the distribution of what nerve?

Answer 34

encephalo-leptomeningeal angiomatosis capillary CN V1 (ophthalmic branch)

Question 35

A baby pops out with a purple mark over his eye/face. The child has a few seizures shortly after birth. What are the main worries for the future, other than those already presented?

Answer 35

glaucoma mental retardation atrophy/calcification of occipital lobe pheochromocytomas

Question 36

How is Sturge-Weber different from other neurocutaneous disorders in relation to its acquisition?

Answer 36

this only occurs SPORADICALLY | All others have AD inheritance

Question 37

Many patients with polyarteritis nodosa are seropositive for a certain infection. What are the basic characteristics of the causal organism?

Answer 37

Hepatitis B virus (a Hepadnavirus) Enveloped, DS DNA virus carries Reverse Transcriptase!

Question 38

An IV drug user presents with abdominal pain and bloody shit. On arteriogram his mesenteric artery looks like a string of pearls. If left untreated, what type of cancer is he at most risk of getting?

Answer 38

Hepatocellular carcinoma | PAN pts are often + for HepB, which chronically can be associated with HCC

Question 39

c-ANCA is an autoantibody against?

Answer 39

Proteinase 3

Question 40

HLA-DR3 is associated with what vasculitis?

Answer 40

Giant cell arteritis

Question 41

What is the only vasculitis that can spread to the whole neurovascular bundle?

Answer 41

Buerger's disease | can lead to Raynaud's phenomenon

Question 42

Cyclophosphamide is the drug of choice for polyarteritis nodosa and many of the small vessel vasculitides. What are the side effects?

Answer 42

Hemorrhagic cystitis (mesna can slow this) AML bladder cancer

Question 43

Lo brachial pulse is to Takayasu disease as lo femoral pulse is to ______________

Answer 43

infantile coarctation of aorta | keeping you on your toes bitch!