Vascular Disease 3- Leah (3)- Vasculitis* Flashcards Preview

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Flashcards in Vascular Disease 3- Leah (3)- Vasculitis* Deck (55):
1

Giant Cell Arteritis (GCA): what vessels are involved?

small --> large
esp those in the head (temporal, ophthalmic, vertebral)
occasionally the aorta= giant cell aortitis

2

In what vessels is polyarteritis nodosa observed?

small --> medium
kidneys, heart, liver/ GI
*SPARES LUNGS

3

Kawasaki disease involves what arteries?

small/ large
most commonly the coronaries

4

Churg Strauss involves what vessels?

small
kidney, skin, GI, ocassionally the heart

5

Wegener's granulomatosis effects what vessels?

small and medium
Lung + upper airway*, and renal

6

Microscopic polyangitis effects what vessels?

Small (rarely large)
very wide distribution of lesions throughout body
*Does NOT spare the lung, unlike PAN

7

Thromboangiitis obliterans effects what vessels?

small/ medium
most commonly tibial and radial

8

Which two vaculidities are assc with nodular aneurysms?

-PAN
-Kawasaki

9

Which vasculidity is assc with fragmented neutros?

MPA

10

Which vasculidity is assc with micro-abscesses and gangrene? Where are the micro-abcesses found?

Thromboarteritis Obliterans:
Vasculitis causes multiple thrombi in vessel lumen,
micro-abcesses are found within the thrombi

11

Which four vasculidities are assc with segmental lesions?
What is the significance of this findings?

-GCA
-PAN
-CS
-TO

*Good People Can Tag Team*
*Larger samples (2-3 cm) must be taken when taking biopsies to screen for these conditions

12

In which layer of the vascular wall are granulomas found in giant cell arteritis?

What are two other histo findings associated with this disease?

How often does this disease have giant cells?

-granulomas in media
-intimal thickening
-elastic lamina fragmentation
-giant cells only 75% of the time

13

Polyarteritis nodosa histologic findings: (4)

- fibrinoid necrosis/ transmural segmented lesions
- simultaneous early + late lesions
- fibrous thickening (late lesions)
- aneurysmal nodules (early lesions)

14

Kawasaki histologic findings: (2)

-aneurysms
-transmural lesions and fibrinoid necrosis, but less than in PAN

15

Describe the lesions found in MPA (4)

-fibrinoid necrosis, transmural lesions
-all lesions in the same stage
-lesions typically pauci-immune (no Ig/ complexes)
-possible fragmented neutros

16

In addition to granulomas, fibrinoid necrosis, and transmural lesions found in Churg Strauss... what histo findings may be present? (2)

-eosinophils!!!!
-Also note that granulomas may be EXTRAVASCULAR

17

Special characteristic of granulomas found in thromboarteritis obliterans (2):

Two things patients may present with clinically?

-Fibrotic tissue may involve nerves and veins
-Nerve involvement = *PAINFUL* in late stage disease

*Clinical presentation: cold sensitivity in hands, instep claudication

18

Signs/ Symptoms of Giant Cell Arteritis (2):
What population is this seen in?
Treatment?

-elevated inflammatory markers
-palpable, painful, nodular vessels + H/A, blindness
-elderly esp in US + Europe (#1 elderly vasculitis)
-Give corticosteroids *MEDICAL EMERGENCY*

19

Findings (Classic Triad+2) + Cause of Death assc with PAN:
Age group assn?

TRIAD:
1. rapid accelerating HTN
2. abdominal Pain
3. bloody stool
-ruptured aneurysms
-decreased perfusion injuries (ulceration, infarcts etc)

*CAUSE OF DEATH = renal vasculitis + malignant HTN (no glomerulonephritis!)
*young adults

20

Treatment for PAN:

-corticosteroids, cyclosporine ++ treat the HTN

21

Kawasaki Disease:
symptoms+ cause of death, course of disease, age group, treatment

CAUSE OF DEATH = ruptured aneurysms and MI
"CRASH and BURN"
-Conjunctivitis
-Rash (lips, palms, soles)
-Adenopathy (cervical, unilateral)
-Strawberry tongue
-Hand + foot edema/erythema
-Burn = FEVER

Tx: ASN and IVIG
*Kids under 4yoa, classically Asians (Hence KAWASAKI!)

22

Churg Strauss key findings: (4)

-eosinophils
-asthma
-allergic rhinitis
-less than 50% will be MPO-ANCA +

23

Wegener's Disease key findings + population: (3)

-PR3 ANCA + (95%)
-chronic pneumonitis, sinusitis, and renal disease
-middle age males most common (+40yoa)

24

Signs and symptoms of thromboarteritis obliterans (4) + population in which it is seen?

1. nodular phlebitis/ chronic ulceration/ gangrene in hands and feet
2. instep claudication
3. cold sensitivity
4. severe pain in late stage
*young adult male smokers most commonly

25

Contrast PAN/ MPA: (6)

-PAN spares the lungs, MPA doesn't
-PAN may have lesions in multiple stages, MPA are all the same stage
-MPA is MPO-ANCA + in most cases, PAN = No ANCA
-MPA = smaller vessels than PAN
-MPA = fragemented neutros
-PAN = aneuryms

26

Asthma, eos, and rhinitis should be a red flag for?

Churg Strauss

27

Pneumonitis, sinusitis, and renal disease should be a red flag for?

Wegner's disease

28

Which disease is assc with Hep B?

PAN- 30% of cases

29

Pauci Immune lesions are assc with?

MPA, lesions are due to immune complex but the complex isnt seen on biopsy

30

Three vasculidities that are specifically assc with middle aged people/ young adults

-PAN
-Wegners
-TO

31

Symptoms of vasculitis in general:

fever, myalgias, arthralgias, malaise
non specific, flu like

32

Three general vasculitis mechanisms:

1. Ab/Ag complex deposits
(drug hypersensitivity, viral infxn.)

2. specific ABs (ANCAs)

3. infectious agents (rare)

33

p-ANCA currently referred to as...

Anti-myeloperoxidase (MPO)-ANCA
-perinuclear antineutrophil cytoplasmic antibody
Assc with MPA/ some CS cases

p --> (mPo)

34

c-ANCA durrently referred to as...

Anti-proteinase-3 (PR3)-ANCA
-cytoplasmic antineutrophil cytoplasmic antibody
Assc with Wegners

c=cytoplasm= "P"eriphery= "P"r3

35

#1 Vasculitis in elderly of US and Europe?

Giant cell arteritis

36

Percent of patients with giant cell arteritis that have ocular sx.?

50%

37

Possible causes of MPA (4)

Ab response to the following:
- drugs
- microorganism
- Heterologous proteins
- tumor Ag's

38

MPA Signs/ Sx. (3)

Determined by vascular bed involvement:
- Reps/ Renal: Hemoptysis, hematuria, proteinuria
- GI: Bowel pain/ bleed
- Skin: palpable purpura

39

Disease asstd. with this clinical triad
1. respiratory disease
2. Vasculitis (small/ medium vessels)
3. renal disease (focal necrotizing glomerulonephritis

Wegener's Granulomatosis

40

Vasculitis with neuro involvement/ neuritis (2)

PAN, TO

41

Disease mechanism for:
Polyarteritis nodosa (PAN) ?

30% assc with (chronic) Hep B Ag/Ab complexes

42

Disease mechanism for:
Microscopic Polyangitis (MPA)?

MPO-ANCA + (P-ANCA +)

43

Disease mechanism for:
Churg Strauss (CS)?

50% cases MPO ANCA+ (P-ANCA +)

44

Disease mechanism for:
Wegener's (WPWG) ?

PR3 ANCA + (C-ANCA +)
- good marker of disease activity

45

Disease mechanism for:
Thromboangitis obliterans (TO)?

tobacco hypersensitivity

46

What 4 conditions are assc with granulomatous lesions?

-GCA
-Wegeners
-thromboangitis obliterans
-churg strauss (intra/extravascular granulomas and ALSO fibrinoid necrosis)

47

What four conditions are assc with fibrinoid necrosis + intimal --> transmural lesions?

-PAN, Kawasaki, MPA= fibrinoid necrosis/ transmural lesions
-Also, Churg Strauss has fibrinoid/ transmural PLUS granulomas

48

How to differentiate takayasu arteritis from giant cell arteritis (2):

1. Takyasu = young adult females (vs. females 50+ yoa)
2. Takyasu = proximal lesions, close to aortic arch (vs. distal arteries of head and neck)

49

What causes Henoch Shonlein Purpura?
How does it present?
How common is this?

- IgA immune complex deposition following URI
- Presents with palpable purport on buttocks and legs, IgA nephropathy, GI bleeds
- #1 vasculitis in kiddos

50

Which condition is associated with intra and extravascular granulomas?

churg strauss

51

Which condition has fibrinoid necrosis/ intimal-->transmural lesions and also has granulomas?

churg strauss

52

Eosinophilic granulomatosis with polyangitis is the same thing as?

Churg Strauss

53

Granulomatosis with polyangitis is another name for?

Wegner's Disease

54

Buerger Disease is the same as?

Thromboangitis obliterans

If Jeff, the hamburler, smokes, his feet will rot off.

55

Three forms of Vasculitis that may have "palpable purpura"?

-Henoch- Schonlein
-Microscopic Polyangitis
-Churg Strauss/ Eosinophilic Granulomatosis with polyangitis