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Nelson - Rheumatic Diseases of Childhood > Vasculitis Syndromes > Flashcards

Vasculitis Syndromes Flashcards

1
Q

Small vessels

A

Capillaries, arterioles, postcapillary venules

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2
Q

Medium vessels

A

Renal arteries, mesenteric vasculature, coronary arteries

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3
Q

Large vessels

A

Aorta and its proximal branches

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4
Q

ANCA association: cANCA

A

1) Wegener granulomatosis 2) Churg-Strauss

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5
Q

ANCA association: pANCA

A

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

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6
Q

ANCA association: ANCA

A

Cystic fibrosis

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7
Q

Predominantly large vessel vasculitis

A

Takayasu arteritis

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8
Q

Predominantly medium vessel vasculitis

A

1) PAN 2) Kawasaki disease

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9
Q

Predominantly small vessel vasculitis

A

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

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10
Q

Granulomatosis with polyangiitis is aka

A

Wegener granulomatosis

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11
Q

Eosinophilic granulomatosis with polyangiitis is aka

A

Churg-Strauss syndrome

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12
Q

HSP affects more males vs females

A

Males

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13
Q

Approx 90% of HSP cases occur in what age group

A

Children, usually between 3 and 10y/o

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14
Q

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

A

T

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15
Q

Common finding in biopsy of HSP is deposition of IgA particularly

A

IgA1

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16
Q

Hallmark of HSP

A

Palpable purpura

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17
Q

Skin lesions of HSP are usually of what distribution

A

Symmetric and in gravity-dependent areas or pressure points

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18
Q

Skin lesions of HSP typically last for how long

A

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

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19
Q

Arthritis and arthralgias occur in ___% of children with HSP

A

75

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20
Q

Arthritis in HSP is usually

A

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

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21
Q

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

A

80

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22
Q

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

A

50

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23
Q

HSP ACR classification criteria

A

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

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24
Q

HSP EULAR criteria

A

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

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25
Q

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

A

Acute hemorhhagic edema (AHE)

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26
Q

Distinguishes AHE from HSP

A

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

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27
Q

T/F Platelet count in HSP is low

A

F, Normal

28
Q

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

A

1) Bowel wall edema 2) Intussusception (rarely)

29
Q

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

A

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

30
Q

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

A

T

31
Q

Major long term complication of HSP

A

Renal disease

32
Q

Renal disease in HSP can develop up to ___ after diagnosis

A

6 months, but rarely happens if initial UA is normal

33
Q

Self-limited course of HSP usually lasts ___ on average

A

4 weeks

34
Q

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

A

4-6 mo

35
Q

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

A

T

36
Q

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

A

T

37
Q

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

A

T

38
Q

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

A

8

39
Q

AKA pulseless disease

A

Takayasu arteritis

40
Q

Takayasu arteritis predominantly affects males vs females

A

Females

41
Q

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

A

Occlusive complications

42
Q

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

A

Aneurysms

43
Q

MC aortic branches affected by takayasu arteritis

A

1) Subclavian 2) Renal 3) Carotid

44
Q

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

A

> 10

45
Q

Mainstay of therapy of Takayasu arteritis

A

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

46
Q

T/F Cutaneous PAN is limited to the skin

A

T

47
Q

Vessel involvement in PAN is usually

A

Segmental and tends to occur at vessel bifurcations

48
Q

Vascular bed that is usually spared in PAN

A

Pulmonary vasculature

49
Q

Gold standard diagnostic imaging study for PAN

A

Conventional arteriography

50
Q

Conventional arteriography in PAN reveals

A

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

51
Q

Mainstay of therapy in PAN

A

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

52
Q

GPA and MPA involve what vascular beds

A

Respiratory tract and kidneys

53
Q

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

A

CSS

54
Q

ANCA-associated vasculitis that can produce a saddle nose deformity

A

GPA

55
Q

Small vessel vasculitis: IgA-dominant immune deposits

A

HSP

56
Q

Small vessel vasculitis: Necortizing vasculitis

A

GPA, CSS, and MPA

57
Q

Small vessel vasculitis: Granulomatous inflammation

A

GPA and CSS

58
Q

Small cell vasculitis: Asthma and eosinophilia

A

CSS

59
Q

Predominantly small vessel vasculitis, granulomatous

A

1) Wegener granulomatosis 2) Churg-Strauss

60
Q

Predominantly small vessel vasculitis, nongranulomatous

A

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

61
Q

MC vasculitis of childhood

A

HSP

62
Q

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

A

HSP

63
Q

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

A

Vasa vasorum

64
Q

Proposed classification criteria for pediatric Takayasu arteritis

A

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
Q

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

A

T

66
Q

Gold standard to establish large vessel arterial involvement in Takayasu arteritis

A

Conventional arteriography of the aorta and its major branches

Nelson - Rheumatic Diseases of Childhood (9 decks)

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