vasculidities Flashcards Preview

STEP 1 > vasculidities > Flashcards

Flashcards in vasculidities Deck (61):
1

what are the key features of microscopic polyangitis

-affects kidneys
-causes pauci-immune crescentic glomerulonephritis
-p-ANCA+

2

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

perinuclear anti-neurophil cytoplasmic antibodies

antigen is myeloperoxidase
antibody is MPO-ANCA

3

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

cytoplasmic anti-nutrophil cytoplasmic antibodies

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

4

what are the key features of granulomatosis with polyangitis

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

5

what is the treatment for granulomatosis with polyangitis?

cyclophosphamide and corticosteroids

6

what are the key features of churg-strauss syndome?

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

7

what are the key features of henoch-schonlein purpura?

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)!!

8

what are the key features of polyarteritis nodosa?

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

9

what is mucocutaneous lymph node syndrome?

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

10

what is a major concern for kawasaki diase?

coronary aneurism

can lead to thrombosis and MI

be prepared to make ID from coronary angiogram

11

what is treatment for kawasaki disease?

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

12

What is another name for Burger disease?

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

13

what is another name for giant cell arteritis?

temporal arteritis

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

14

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

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

15

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

temporal arteritis

16

what's another name for takiyatsi disease?

pulseless disease

due to granulomatous inflamation of arteries of the aortic arch

17

what are the key features of takiyatsi arteritis?

young asian women in teen

poor pulses in extemities

granulomatous inflamation of arteries of the aortic arch

elevated sed rate

18

vasculitis is 2 year old asian female

kawasaki

19

vasculitis in 20 year old asean female

takiyatsu

20

vasculitis associated with asthma

churg strause

21

most likely vasculitis in young male smoker

burger (thromboarteritis obliterans)

22

vasculitis associated with polymylagia rheumatica

temporal arteritis

23

vasculitis associated with IgA nephropatrhy

henoch-schloch purpura

24

vasculitis associated with Hep B

polyarteritis nodosa

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

Decks in STEP 1 Class (77):