Vasculitis Syndromes Flashcards Preview

Nelson - Rheumatic Diseases of Childhood > Vasculitis Syndromes > Flashcards

Flashcards in Vasculitis Syndromes Deck (66):
1

Small vessels

Capillaries, arterioles, postcapillary venules

2

Medium vessels

Renal arteries, mesenteric vasculature, coronary arteries

3

Large vessels

Aorta and its proximal branches

4

ANCA association: cANCA

1) Wegener granulomatosis 2) Churg-Strauss

5

ANCA association: pANCA

1) Microscopic polyarteritis 2) Ulcerative colitis 3) Sclerosing cholangitis 4) Crohn disease %) Autoimmune hepatitis type 1

6

ANCA association: ANCA

Cystic fibrosis

7

Predominantly large vessel vasculitis

Takayasu arteritis

8

Predominantly medium vessel vasculitis

1) PAN 2) Kawasaki disease

9

Predominantly small vessel vasculitis

1) Granulomatosis with polyangiitis 2) Eosinophilic granulomatosis with polyangiitis 3) Microscopic polyangiitis 4) HSP 5) Isolated cutaneous luekocytoclastic vasculitis 6) Hypocomplementemic urticarial vasculitis

10

Granulomatosis with polyangiitis is aka

Wegener granulomatosis

11

Eosinophilic granulomatosis with polyangiitis is aka

Churg-Strauss syndrome

12

HSP affects more males vs females

Males

13

Approx 90% of HSP cases occur in what age group

Children, usually between 3 and 10y/o

14

T/F HSP in adults present with more severe and chronic complications

T

15

Common finding in biopsy of HSP is deposition of IgA particularly

IgA1

16

Hallmark of HSP

Palpable purpura

17

Skin lesions of HSP are usually of what distribution

Symmetric and in gravity-dependent areas or pressure points

18

Skin lesions of HSP typically last for how long

3-10 days and may recur up to 4 months after initial presentation

19

Arthritis and arthralgias occur in ___% of children with HSP

75

20

Arthritis in HSP is usually

1) Oligoarticular 2) Predilection for lower extremities 3) Does not lead to deformities 4) Usually resolves in 2 weeks

21

GI manifestations of HSP occur in up to ___% of patients with HSP

80

22

Renal involvement occurs in up to ___% of children with HSP

50

23

HSP ACR classification criteria

2 of the ff must be present 1) Palpable purpura 2) Age at onset less than or equal to 20 y/o 3) Bowel angina (postprandial abdominal pain, bloody diarrhea) 4) Biopsy demonstrating intramural granulocytes in small arterioles and/or venules

24

HSP EULAR criteria

1 or more must be present 1) Abdominal pain 2) Arthiris or arthralgia 3) Biopsy of affected tissue demonstrating predominant IgA deposition 4) Renal involvement (proteinuria >3g/24hr), hematuria or red cell casts

25

An isolated cutaneous leukocytoclastic vasculitis that affects infants <2 yr of age, that resembles HSP clinically

Acute hemorhhagic edema (AHE)

26

Distinguishes AHE from HSP

1) Younger age 2) Nature of the lesions 3) absence of other organ involvement 4) biopsy

27

T/F Platelet count in HSP is low

F, Normal

28

UTZ is done in patients with HSP and GI symptoms to look for

1) Bowel wall edema 2) Intussusception (rarely)

29

___ are most often used to treat significant GI involvement and joint pain in HSP

Steroids (Prednisone 1mkday for 1-2 weeks followed by taper)

30

T/F Prednisone in HSP does not alter overall prognosis nor prevent renal disease

T

31

Major long term complication of HSP

Renal disease

32

Renal disease in HSP can develop up to ___ after diagnosis

6 months, but rarely happens if initial UA is normal

33

Self-limited course of HSP usually lasts ___ on average

4 weeks

34

From 15-60% of children with HSP experience 1 or more recurrences, typically within ___ of diagnosis

4-6 mo

35

T/F HSP: With each relapse, symptoms are usually milder than at presentation

T

36

T/F HSP: Children with a more-severe initial course are at higher risk for relapse

T

37

T/F HSP: The long-term prognosis usually depends upon the severity and duration of gastrointestinal or renal involvement

T

38

Approximately ___% of those with HSP nephritis go on to have end-stage renal disease

8

39

AKA pulseless disease

Takayasu arteritis

40

Takayasu arteritis predominantly affects males vs females

Females

41

Complication of takayasu arteritis that is more common in United States, Western Europe, and Japan

Occlusive complications

42

Complication of takayasu arteritis that is more common in SEA and Africa

Aneurysms

43

MC aortic branches affected by takayasu arteritis

1) Subclavian 2) Renal 3) Carotid

44

___mmHg difference in systolic BP among 4 extremities is indicative of disease

>10

45

Mainstay of therapy of Takayasu arteritis

Glucocorticoids, starting with high doses (Pred 1-2mkday)

46

T/F Cutaneous PAN is limited to the skin

T

47

Vessel involvement in PAN is usually

Segmental and tends to occur at vessel bifurcations

48

Vascular bed that is usually spared in PAN

Pulmonary vasculature

49

Gold standard diagnostic imaging study for PAN

Conventional arteriography

50

Conventional arteriography in PAN reveals

"Beads on a string" pattern (areas of aneurysmal dilation and segmental stenosis)

51

Mainstay of therapy in PAN

Oral (1-2mkday) and IV pulse (30mkday) prednisone

52

GPA and MPA involve what vascular beds

Respiratory tract and kidneys

53

A small vessel necrotizing granulomatous (allergic granulomatosis) vasculitis associated with a history of refractory asthma and peripheral eosinophilia

CSS

54

ANCA-associated vasculitis that can produce a saddle nose deformity

GPA

55

Small vessel vasculitis: IgA-dominant immune deposits

HSP

56

Small vessel vasculitis: Necortizing vasculitis

GPA, CSS, and MPA

57

Small vessel vasculitis: Granulomatous inflammation

GPA and CSS

58

Small cell vasculitis: Asthma and eosinophilia

CSS

59

Predominantly small vessel vasculitis, granulomatous

1) Wegener granulomatosis 2) Churg-Strauss

60

Predominantly small vessel vasculitis, nongranulomatous

1) Microscopic polyangitis 2) HSP 3) Isolated cutaneous leukocytoclastic vasculitis 4) Hypocomplementemic urticarial vasculitis

61

MC vasculitis of childhood

HSP

62

Characterized by leukocytoclastic vasculitis and IgA deposition in small vessels of the skin, joints, GIT, and kidneys

HSP

63

Takayasu arteritis is characterized by inflammation of the vessel wall starting at what layer

Vasa vasorum

64

Proposed classification criteria for pediatric Takayasu arteritis

Angiographic abnormalities of the aorta or its main branches AND at least one of the ff: 1) Decreased peripheral artery pulse and/or claudication of extremities 2) BBP diff between arms and legs of >10mmHg 3) bruits over the aorta and/or its major branches 4) Htn

65

T/F Radiographic assessment is essential to establish large vessel arterial involvement

T

66

Gold standard to establish large vessel arterial involvement in Takayasu arteritis

Conventional arteriography of the aorta and its major branches