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vasculidities Flashcards

1
Q

what are the key features of microscopic polyangitis

A
  • affects kidneys
  • causes pauci-immune crescentic glomerulonephritis
  • p-ANCA+
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2
Q

what is p-ANCA? What is the antigen and what is the antibody?

A

perinuclear anti-neurophil cytoplasmic antibodies

antigen is myeloperoxidase
antibody is MPO-ANCA

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3
Q

what is c-ANCA? What is the antigen and what is the antibody?

A

cytoplasmic anti-nutrophil cytoplasmic antibodies

antigen is proteinase 3 (PR3)
antibody is PR3-ANCA

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4
Q

what are the key features of granulomatosis with polyangitis

A

focal necrotizing vasculitis with granulomas in the lung and upper airway
glomerulonephritis
c-ANCA

NOTE:differentiate from goodpastures by involvement of upper airway in wegners

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5
Q

what is the treatment for granulomatosis with polyangitis?

A

cyclophosphamide and corticosteroids

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6
Q

what are the key features of churg-strauss syndome?

A

granulomatosis with eosinophilia (multinucleated giant cells with eosinophils) and ASTHMA/sinusitis/skin lesions/peripheral neuropathy (foot drop/wrist drop)
+pANCA

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7
Q

what are the key features of henoch-schonlein purpura?

A

most-common form of systemic vasculitis in childhood

  • rash on buttocks and legs made up of PALPABLE PUPURA
  • arthralgias (knees)
  • abdominal pain with melana(intestinal hemorrhage)
  • renal disease (deposition of IgA immune complexes - IgA nephropathy) with nephritic hematuria

OFTEN OCCURS FOLLOWING UPPER RESPIRATORY INFECTION (IgA nephropathy)!!

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8
Q

what are the key features of polyarteritis nodosa?

A

medium vessel vasculitis that involves kidneys and visceral organs but spares lungs - can have abdominal pain melana, myalgia

associiated with hepB/hepC

NEGATIVE FOR ANCA

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9
Q

what is mucocutaneous lymph node syndrome?

A

kawasaki’s disease
necrotizing vasculitis seen in asiean infants
fever that lasts >5 days + CRASH symptoms

conjunctuvitis
rash on trunk
adenopathy
strawberry tounge
hands and feet - erythematous desquamation
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10
Q

what is a major concern for kawasaki diase?

A

coronary aneurism

can lead to thrombosis and MI

be prepared to make ID from coronary angiogram

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11
Q

what is treatment for kawasaki disease?

A

IV immunoglobulin (goal is to flood system with immunoglobulin and cause feedback-suppression of immune responses to decrease production of auto-antibodies) and asprin

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12
Q

What is another name for Burger disease?

A

thromboangiitis obliterans

vasculitis of medium arteries seen in male smokers in their 40’s

intermittent claudication
superficial nodular phlebitis
can lead to gangreen in fingers

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13
Q

what is another name for giant cell arteritis?

A

temporal arteritis

affects branches of EXTERNAL carotid arteries (e.g. temporal arteries)

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14
Q

who is predominantly affected by temporal arteritis? what is the classic presentation?

A

classicly presents in elderly women as unilateral headache with jaw-claudication
occlusion of opthalmic artery can lead to vision loss or irriversible blindness

may have palpable temporal artery

may also have polymyalgia rhumatica (muscle and joint pain)

dx is with temporal artery biopsy

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15
Q

pt presents with unilateral headache and jaw pain with chewing and elevated ESR. What is Dx?

A

temporal arteritis

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16
Q

what’s another name for takiyatsi disease?

A

pulseless disease

due to granulomatous inflamation of arteries of the aortic arch

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17
Q

what are the key features of takiyatsi arteritis?

A

young asian women in teen

poor pulses in extemities

granulomatous inflamation of arteries of the aortic arch

elevated sed rate

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18
Q

vasculitis is 2 year old asian female

A

kawasaki

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19
Q

vasculitis in 20 year old asean female

A

takiyatsu

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20
Q

vasculitis associated with asthma

A

churg strause

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21
Q

most likely vasculitis in young male smoker

A

burger (thromboarteritis obliterans)

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22
Q

vasculitis associated with polymylagia rheumatica

A

temporal arteritis

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23
Q

vasculitis associated with IgA nephropatrhy

A

henoch-schloch purpura

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24
Q

vasculitis associated with Hep B

A

polyarteritis nodosa

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25
vasculitis associated with jaw claudication and vision loss
temporal/giant cell arteritis
26
vasculitis associated with strawberry tongue
kawasaki's disease
27
vasculitis associated with desquamation of hands/feet
kawasaki's disease
28
vasculitis associated with poor pulses in the arms
takiyasu arteritis
29
vasculitis associated with palpable purpura on the legs
henoch-schlochen purpura
30
vasculitis associated with kidney and GI involvement but spares the lungs
polyarteruitis nodosa HEPATITIS!!!
31
vasculitis of the kidney, upper airway and lungs
granulomatosis with polyangitis
32
what's the treatment for renauds?
dihydropyridine calcium channel blockers (e.g. nefidipine)
33
what is sturge weber disease?
congential disorder that affects capillaries causing AV malformation princply presents as leptomeningeal angioma purplish port-wine stain along opthalmic division of trigeminal nerve associated with seizures, glocoma, tardism
34
what is a strawberry hemangioma?
benign hemanioma of cutaneous suface of capillaries seen in infants - spontaneous regresses in childhood (gone by age 8)
35
what is a cherry hemanioma?
benign hemanioma of cutaneous surface capillaries - does not regress very common on most people
36
what is a pyogenic granulmoma>
raised capillary hemanioma associated with trauam and pregnancy looks like red-polyp on skkin - frequently ulcerates and bleeds and tend to be on lips an gums
37
what is a glomus tumor?
benign painful tumor under the fingernails
38
what is bacillary angiomatosis? what is it associated with?
benign but can be mistaken for karposi sarcoma because looks like multiple raised puplish spots caused by bartonella henselae - cat scratch disease in immunocompromised
39
what vascular tumor is associated with prior radiation therapy?
angiosarcoma - often face, scalp, neck or breast
40
what is a potential consequence of perisisten lymphadema?
lymphangiosarcoma
41
vasculitis associated with weak pulses in upper extrmeities
takiyatsu
42
vasculitis associated with necrotizing granulomas of lung and necrotizing glomerulonephritis
granulomatosis with polyangitis
43
vasculitis associated with necrotizing immune complex inflammation of viseral/renal vessels
``` polyarteritis nodosa (spares lungs, -ANCA) ```
44
vasculitis associated with young male smokers
buerger ddisease
45
vasculitis associated with young asian women
takayasu disease
46
vasculitis associated with young asthmatics
churg-strauss
47
vasculitis associated with infants and young children; involves coronary arteries
kawasaki
48
most common vasculitis
temporal arterisit
49
vasculitis associated with hep B
polyarteritis nodosa
50
vasculitis associated with occlusion of opthalamic artery that can lead to blindness
temporal arteritis
51
vasculitis associated with perforation of the nasal septum
wegners
52
vasculitis associated with unilateral headache with jaw claudication
temporal arteritis
53
benign, raised, red lesion about the size of a mole in older patients
cherry hemangioma
54
raised, red area present at birth increases in size initially then regresses over months to years
strawberry angioma
55
lesion caused by lymphoangiogenic growth factors in HIV patient
karposi-sarcoma
56
polypoid red lesion found in prgnancy or after trauma
pyogenic angioma
57
benign, painful red-blue tumor under fingernailos
glomus tumor
58
cavernous lymphangioma associated with turner syndromwe
cystic hygroma
59
skin papule in AIDS patients caused by bartonella
bacilliary angiomatosis
60
what is the treatment for bueger disease?
smoking cessation
61
what is the trwatment for temporal arteritis
high dose steroids

STEP 1 (77 decks)

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