Vasculitis Flashcards

1
Q

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

A

exposure of subendothelial collagen (thrombus)

inflammation/fibrosis = stenosis

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2
Q

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

A

Temporal/giant cell arteritis

Takayasu arteritis

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3
Q

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

A

Asian females < 40

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4
Q

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

A

aortic arch

branches of carotid

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5
Q

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

A

BOTH Giant Cell and Takayasu!

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6
Q

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

A

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

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7
Q

Which large vessel vasculitis can be associated with polymyalgia rheumatica?

A

Giant cell arteritis

GCA+fever, malaise, myalgia, arthralgia

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8
Q

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

A

similar patients!

Giant Cell: painless blindness
MS: painful blindness

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9
Q

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

A

most likely young (Asian)

describes ‘pulseless disease’; aka Takayasu arteritis

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10
Q

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?

A

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

Giant Cell arteritis is FOCAL (can skip areas)

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11
Q

The three Medium vessel vasculitis disorders to know are?

A

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

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12
Q

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

A

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

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13
Q

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

A

Polyarteritis nodosa

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14
Q

__________ is a medium vessel vasculitis that involves the digits

A

Buerger’s disease

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15
Q

__________ is a medium vessel vasculitis involving the Coronary artery

A

Kawasaki disease

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16
Q

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

A

IVIG + ASA (say whaaaaaaat)

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17
Q

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

A

Reye’s syndrome

stop clot formation

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18
Q

How do patients with Kawasaki disease present?

A

Non-specfic symptoms, sounds infectious!

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

19
Q

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

A

many arteries (except lung)

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

20
Q

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

A

type III HS

seropositive for Hep B (HBsAg)

21
Q

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

A

Corticosteroids or Cyclophosphamide

tx for Polyarteritis nodosa

22
Q

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 ________

A

smoking

autoamputation

23
Q

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

A

ANCA (anti-neutrophilic antibody)

24
Q

Compare the ANCA positivities in small vessel vasculitides.

A

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

25
Q

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

A

MP: NO granulomas + NO asthma + NO Eosinophilia + lung/kidney

CSS: Granulomas + asthma + Eosinophilia + lung/heart

26
Q

What are the key 3 areas involved with Wegener granulomatosis?

A

URT
LRT
Renal

27
Q

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

A

MP: NO granulomas + p-ANCA + NO URT involvement

WG: Granulomas + c-ANCA + URT involvement

28
Q

What is the tx for the 3 ANCA small vessel vasculitides

A

Cyclophosphamide or Corticosteroids

29
Q

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

A

upper respiratory tract infection in kids

30
Q

HSP classically presents as what 4 things?

A

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

31
Q

What characteristic lab is found in HSP?

A

hi serum IgA (complexes cause dz)

32
Q

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

A

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)

33
Q

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?

A

c-ANCA (Wegener)

vs p-ANCA (microscopic polyangiitis)

34
Q

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

A

encephalo-leptomeningeal angiomatosis
capillary
CN V1 (ophthalmic branch)

35
Q

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?

A

glaucoma
mental retardation
atrophy/calcification of occipital lobe
pheochromocytomas

36
Q

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

A

this only occurs SPORADICALLY

All others have AD inheritance

37
Q

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

A

Hepatitis B virus (a Hepadnavirus)

Enveloped, DS DNA virus

carries Reverse Transcriptase!

38
Q

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?

A

Hepatocellular carcinoma

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

39
Q

c-ANCA is an autoantibody against?

A

Proteinase 3

40
Q

HLA-DR3 is associated with what vasculitis?

A

Giant cell arteritis

41
Q

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

A

Buerger’s disease

can lead to Raynaud’s phenomenon

42
Q

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

A

Hemorrhagic cystitis (mesna can slow this)
AML
bladder cancer

43
Q

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

A

infantile coarctation of aorta

keeping you on your toes bitch!