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Flashcards in Vasculitis Deck (49):
1

What are some symptoms common to vasculitis?

- palpable purpura (non-blanching)
- livedo reticularis (due to dilation of blood vessels)
- urticaria

VERY variable however

2

What would a blanching lesion indicate?

blood vessel swelling because you can physically push the blood out

3

What are the two major cause of vasculitis?

- immune mediated inflammation (most)

- direction invasion of vascular walls by infectious pathogens

4

What else can infections do?

cause a noninfectious vasculitis, by generating immune complexes or triggering a cross-reactive immune response

5

Immunologic injury in non-infectious vasculitis may be caused by:

- immune complex deposition
- anti-neutrophil antibodies (ANCA)
- antiendothelial cell antibodies
- autoreactive T cells

6

What are some vasculitis' that affect large vessels (aorta and its primary branches)?

- temporal (giant cell) arteritis
- Takayasu arteritis

7

What are some vasculitis' that affect medium vessels (muscular arteries and supply organs)?

- Polyarteritis nodosa
- Kawasaki Disease
- Buerger disease

8

What are some vasculitis' that affect small vessels (arterioles, capillaries, and venules)?

- Wegener's Granulomatosis
- Mircoscopic polynagiitis
- Churg-strauss syndrome
- Behcet disease
- Henoch-Scholein purpura
- Cyroglobulinemia

9

How do temporal arteritis (TA) patients present?

- older (50+), white females
- jaw pain when they chew
- ESR often elevated over 100

10

What is the etiology of TA?

T cell mediated

11

What is the treatment for TA?

steroids

12

What is the major complication of TA?

blindness- there is patchy ophthalmic artery involvement. Thus, treatment is started before diagnosis usually

13

What happens in TA?

- intima thickening, - inflammation of ALL 3 layers of the vessel

14

Half of patients with TA also have what?

polymyalgia rheumatica

15

What is polymyalgia rheumatica?

- proximal muscle aches and stiffness
- elevated ESR
- rapid resolution of symptoms with low-dose corticosteroid therapy

16

What is Takayasu arteritis?

granulomatous vasculitis (like TA- hist looks exactly the same) that classical involves the aortic arch at branch points and is common adults LESS than 50 y/o (primarily young, female Asians)

17

How do Takayasu arteritis patients present?

-younger than 50 yo
-visual and neurologic symptoms with weak/absent upper extremity pulse
-ESR elevated

18

Treatment for Takayasu arteritis?

-steroids
-prognosis isn't that good

19

How does Polyarteritis nods (PAN) present?

-younger adults
-typically episodic
-systemic inflammation, not localized

20

What organ is especially targeted by PAN?

renal involvement is the major cause of death

PAN is fatal unless treated with immunosuppression

21

PAN is associated with what?

strong association with hepatitis B

22

How can you differentiate PAN from Wegener's?

in PAN, the lungs are essentially never affected

23

How does PAN appear histo wise?

-segmental transmural necrotizing inflammation of small to medium sized arteries (can lead to aneurysm)

-fibrinoid necrosis is common

24

How does Kawasaki disease present?

-affects mostly Asian kids

-CRASH and burn:
Conjunctivitis,
Rash,
Adenopathy (cervical),
Strawberry tongue,
Hand or feet changes/swelling/desquamation,
burn (fever 5+ days)

25

What does untreated Kawasaki disease lead to?

coronary aneurysm and MI

26

Treatment for Kawasaki disease?

-IVIg
-Aspirin (the only time you give aspirin)

27

Mircoscopically Kawasaki disease resembles what?

PAN

28

What is thromboangiitis Obliterates (Buerger Disease)?

a disease of younger smokers that causes inflammation and thrombosis of the arteries supplying the hands and feet, leading to claudication, cold sensitivity, ischemic pain, and eventually gangrene with autoamputation of digits

29

What is the treatment for Buerger disease?

cessation of smoking

30

What do the thrombi in Buerger disease contain?

small micro abscesses composed of neutrophils surrounded by granulomatous inflammation

the thrombus may eventually organize and recanalize

31

What is Raynaud phenomenon?

inflammation and vasoconstriction in response to cold or stress affecting the hands and feet leading to white digits (lack of blood flow), processing to blue color (cyanosis) and finally red (reactive hyperemia when blood flow is restored). Common in young women

32

How is Raynaud's phenomenon treated?

calcium channel blockers and avoiding cold

33

How does Granulomatosis with Polyangiitis (Wegener's) present? Triad?

- affects children and adolescents mostly
triad: granulomas, vasculitis, glomerulonephritis

34

What is a marker of GP?

c-ANCA (PR3-ANCA)- likely directly activates neutrophils to release oxygen radicals and proteolytic enzymes causing endothelial damage

35

How is GP treated?

cyclophosphamide and corticosteroids

36

What are some classical clinical findings of GP?

-chronic sinusitis with nasal petal perforation (saddle nose deformity), nosebleeds

- hemoptysis from lung involvement

- hematuria from renal involvement

37

How does microscopic polyangiitis (MP) present microscopically?

palpable purpura

similar to Wegener with lung and kidney involvement but there are no granulomas

lesions are all the same age, unlike PAN

38

What antibody is MP associated with?

MPO-ANCA

39

What is Churg Strauss Syndrome?

affects those with allergies/asthma (aka allergic granulomatosis and agnates)

40

CSS is associated with what?

-MPO-ANCA
-**eosinophilia**

41

What is Behcet disease?

clinical triad of recurrent oral aphthous ulcers, genital ulcers, and uveitis

42

How is Behcet treated?

immunosuppression with steroids or TNF-anatognists

43

What is the triad for Henoch-Schonlein Purpura?

-palpable purpura on legs and butt from inflamed vessels
-arthritis
-abdominal pain and blood stool
-renal disease

can lead to intussusception because swelling can act as a lead point to drag itself into the adjacent loop of bowel

common in kids

44

What does HSP follow usually?

viral upper respiratory infection

45

What causes HSP?

IgA immune complex deposition in mesangium capillaries (more benign than IgA nephropathy)

46

What is cryoglobulemia?

affects adults with hep C and these are proteins that precipitate in cold but redissolve with warming that when deposited causing inflammation and damage

47

What is infectious vasculitis?

direct invasion of infectious agents into vessel walls or can arise from hematogenous spread of microorganisms during septicemia or embolization from infective endocarditis

48

What agents commonly cause infectious vasculitis?

-usually bacteria (Pseudomonas))
-or fungi (Aspergillus and Mucor species)- common in diabetics

49

What can infectious vasculitis lead to?

can weaken arterial walls and culminate in mycotic aneurysms or induce thrombus and infarction