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Flashcards in Vasculitis (Postlew) Deck (54):
1

Primary finding in vasculitis

inflammation within blood vessel walls

2

What arteries does Polyarteritis Nodosa affect?

medium-sized
(spares arterioles, venules, capillaries)

3

Polyarteritis Nodosa is associated with...

Hep B (30% of patients)

4

Polyarteritis Nodosa: epidemiology

slight male predominance

5

Polyarteritis Nodosa: Clinical Manifestations

--neuropathy
--kidney involvement (= HTN and renal failure)
--testicular tenderness
--cardiac involvement (MI, CHF, pericarditis)
--skin (livedo reticularis)

6

Polyarteritis Nodosa: lab findings

↑ESR, ↑CRP, hematuria, ↑creatinine

7

Cutaneous Polyarteritis Nodosum is associated with...

Hep C

8

How is Buerger’s Disease characterized?

Granulomatous inflammation, which predominantly affects vascular supply to the lower limbs

9

Buerger’s Disease: epidemiology

young adult M, tobacco smokers

10

Which CT disorders can be associated with IC-mediated vasculitis?

SLE, Sjögren's syndrome, and RA

11

What is the most prominent feature in Immune Complex-Mediated Small Vessel Vasculitis?

Cutaneous involvement of small blood vessels

12

What is the classic cutaneous symptom associated with Immune Complex-Mediated Small Vessel Vasculitis?

palpable purpura

(pustules, vesicles, urticaria, small ulcerations may also be found)

13

Hypersensitivity Vasculitis usually occurs secondary to...

drug exposures or infections

14

How is Hypersensitivity Vasculitis characterized?

IC deposition in capillaries, postcapillary venules and arterioles

15

Clinical manifestations of Hypersensitivity Vasculitis?

--purpura
--arthritis
--glomerulonephritis
--colicky abdominal pain

16

Henoch-Schonlein Purpura: epidemiology

90% in children

17

What vessels does Henoch-Schonlein Purpura affect?

small vessels, primarily postcapillary venules

18

What is characteristically present in Henoch-Schonlein Purpura?

IgA

19

Henoch-Schonlein Purpura: classic tetrad

purpura, arthritis, abdominal pain, GN

20

Henoch-Schonlein Purpura: lab findings

↑ESR, ↑CRP, hematuria, ↑creatinine, RBC casts present (if renal involvement)

21

Cryoglobulinemia Vasculitis is most often associated with...

long-standing hep C infection

22

How are types II/III of Cryoglobulinemia Vasculitis different than type I?

IgM and IgG (polyclonal)
associated with hep C, Sjogrens, SLE
result in IC-mediated vasculitis

23

Cryoglobulinemia Vasculitis: clinical manifestations

--palpable purpura with ulcers
--systemic symptoms
--neurological involvement, peripheral neuropathy
--Raynaud’s
--Digital ischemia (often secondary to cold_
--Secondary Sjogren’s

24

Cryoglobulinemia Vasculitis: lab findings

↑ESR, ↓C4, (+) RF

25

What happens if heparin is in collection tubes when trying to dx Cryoglobulinemia Vasculitis?

can → false (+) cryofirinogen

26

Cryoglobulinemia Vasculitis: pathology

leukocytoclastic vasculitis with IgM + C3 deposits in/around small and medium sized vessels

27

What vessels does Giant Cell Arteritis (Temporal) affect?

large sized arteries originating from the arch of the aorta

28

How is Giant Cell Arteritis characterized?

Granulomatous inflammation

29

Giant Cell Arteritis: epidemiology

Age >50, Females 3:1, Northern European ancestry

30

Giant Cell Arteritis: potentially serious complication

thoracic aortic aneurysms

31

Clinical Manifestations of GCA?

--Systemic symptoms (fever, weight loss, fatigue)
--Temporal headaches
--Visual disturbances (can progress to sudden blindness; due to ↓blood to ophthalmic a)
--Jaw or tongue claudication
--Arthralgias
--Tender, swollen temporal artery

32

GCA: lab findings?

markedly elevated ESR

33

If you think a patient may have Giant Cell Arteritis, what is the first thing you should do?

begin trx to prevent blindness; do NOT wait for biopsy

34

What vessels does Takayasu’s Arteritis affect?

large-sized arteries + involvement of aortic arch + branches (+ coronary/pulm arteries)

35

How is Takayasu’s Arteritis characterized?

Granulomatous inflammation

36

Takayasu’s Arteritis: epidemiology

adolescent girls and young women of Asian descent

37

What vessels does Kawasaki Disease affect?

small and medium-sized muscular arteries, especially coronary arteries

38

Kawasaki Disease: epidemiology

Children, Asian ancestry

39

Kawasaki Disease is known to cause...

acquired heart disease (**esp in US and Japan)

40

What causes most of the mortality in Kawasaki Disease?

Coronary lesions
(diseases causes pericardial effusion, myocarditis, aneurysms, MI)

41

Clinical Manifestations of Kawasaki Disease?

fever, rash, strawberry tongue, erythema of palms/soles, edema of hands/feet, conjunctival injection...

42

What occurs in the second week of Kawasaki Disease?

profound lymphocytosis

43

Cutaneous vasculitis in Connective Tissue Diseases is associated with...

hypocomplementemia and ↑↑ANAs

44

Pathology associated with Connective Tissue Disease Associated Vasculitis?

IgG and C3 deposition in/around dermal blood vessels +/- IgM

45

What are the hallmarks of Rheumatoid Vasculitis?

--Pupuric lesions +/- concomitant medium vessel vasculitis
--Deep cutaneous ulcer over malleoli

46

Rheumatoid Vasculitis typically occurs in patients with what condition?

nodular, joint-destructive, RF(+) RA

47

Clinical manifestations associated with Microscopic Polyangiitis?

--glomerulonephritis
--lung involvement + pulm hemorrhage
--skin involvement
--systemic symptoms

48

Microscopic Polyangiitis: lab findings

↑ ESR
↑ CRP
RBC casts in urine
↑creatinine
ANCA positive (usually p-ANCA)

49

Granulumatosis With Polyangiitis: Clinical Manifestations (classic triad?)

**ENT, respiratory tract and kidney involvement

glomerulonephritis, sinusitis, nasal ulcerations, saddle nose, +/- pulm infiltrates or nodules

50

Most notable lab finding associated with Granulumatosis with Polyangiitis?

C-ANCA

51

Allergic Granulomatosis With Polyangiitis: lab findings

↑ESR, eosinophilia, eosinophiluria, RBC casts, proteinuria

52

Allergic Granulomatosis With Polyangiitis: Clinical manifestations

Systemic symptoms (fever, weight loss, fatigue)
Lungs: dyspnea, cough
Neurological: peripheral neuropathy
GU: eosinophilic granulomas of urinary tract and/or prostate

53

Bechet’s Disease: clinical manifestations

--Oral and genital ulcers
--ocular lesions
--Cutaneous lesions (pyoderma gangrenousum-like lesions, pathergy lesions)
--arthritis

54

Bechet’s Disease: epidemiology

20’s and 30’s, M=F
(in US/Korea/China F>M, in Middle East M>F)