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Vasculitis > Ch. 33 Immune Complex Small-Vessel Vasculitis: IgA Vasculitis (Henoch-Schönlein) and Hypersensitivity Vasculitis > Flashcards

Ch. 33 Immune Complex Small-Vessel Vasculitis: IgA Vasculitis (Henoch-Schönlein) and Hypersensitivity Vasculitis Flashcards

1
Q

Leukocytoclasis is the predominant inflammatory reaction in which vasculitides

A

1) IgAV
2) Hypersensitivity vasculitis
3) Mixed cryoglobulinemia

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2
Q

Conditions associated with leukocytoclastic vasculitis

A

Box 33.1 (p.457)

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3
Q

MC systemic vasculitis of childhood

A

IgAV

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4
Q

Diagnostic triad of IgAV

A

1) Purpuric rash
2) Arthritis
3) Abn urinary sediments

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5
Q

2010 Classification Criteria for IgAV

A

Palpable purpura (mandatory) with LE predominance unrelated to thrombocytopenia + 1/4
1) Diffuse abdominal pain
2) Histopath: Skin (LCV) or kidney (proliferative GN with preominant IgA deposition)
3) Arthritis/arthralgias
4) Renal involvement (hematuria and/or proteinuria)

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6
Q

T/F Atypical distribution of purpura in suspected IgAV requires biopsy

A

T

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7
Q

T/F IgAV is more severe when it occurs in adults

A

T

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8
Q

IgAV most frequently occurs in what age group

A

3-12y

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9
Q

IgAV is rare in what age

A

<2y

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10
Q

IgAV is more common in what gender

A

M

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11
Q

T/F IgAV is often preceded by an URTI

A

T, 30-50%

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12
Q

T/F IgAV has been associated with exposure to pharmaceutical agents

A

T

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13
Q

T/F There is a strong causative association between vaccinations and vasculitides, including IgAV

A

F

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14
Q

Suggested pathogenesis of IgAV nephritis

A

Galactose-deficient IgA1 (Gd-IgA1) is recognized by antiglycan antibodies, leading to the formation of circulating immune complexes and their mesangial deposition

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15
Q

Most specific and sensitive criterion for IgAV

A

Histopath

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16
Q

Constitutional signs often present in IgAV

A

Low-grade fever or malaise

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17
Q

Presenting symptom in ~3/4 of patients with IgAV

A

Skin manifestations

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18
Q

T/F Ulcerations do not develop in IgAV

A

F, may occasionally develop in large ecchymotic areas

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19
Q

GI symptoms of IgAV usually appears ___

A

Within 1 week after onset of rash and almost always within 30 days

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20
Q

T/F Abdominal pain may precede all other manifestations of IgAV

A

T

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21
Q

Most frequently involved site of IgAV in the GI tract because of its predilection toward ischemic injury

A

Small bowel

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22
Q

MC surgical complication of IgAV

A

Intussusception

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23
Q

GN affects about ___ of children with IgAV and is potentially life-threatening in ___

A

1/3, <10%

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24
Q

T/F Renal disease in IgAV commonly precedes purpura

A

F, seldom

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25
Q

If serious renal disease develops in IgAV, it does so within

A

4-6 weeks of rash onset

26
Q

risk factors for renal involvement in IgAV

A

Male
Older
Severe GI
Arthritis/arthralgia
Persistent purpura
Raised WBC and/or PC
Elevated ASO
Low C3
RElapse

27
Q

Definitions of severity of IgAV nephritis (SHARE): Mild

A

Normal GFR and mild/moderate proteinuria

28
Q

Definitions of severity of IgAV nephritis (SHARE): Moderate

A

<50% crescents
Impaired GFR OR severe presistent proteinuria

29
Q

Definitions of severity of IgAV nephritis (SHARE): Severe

A

> 50% crescents
Impaired GFR OR presistent proteinuria

30
Q

PErsistent proteinuria (UPCr)

A

> 250mg/mmol x 4 weeks
100 x 3 months
50 x 6 months

31
Q

Mild proteinuria (UPCr)

A

<100mg/mmol

31
Q

Moderate proteinuria (UPCr)

A

100-250mg/mmol

32
Q

Severe proteinuria (UPCr)

A

> 250mg/mmol

33
Q

ISKDC histologic classification of IgAV nephritis: Mild IgAV

A

Class I (minimal change) or II (mesangial change)

34
Q

ISKDC histologic classification of IgAV nephritis: Moderate IgAV

A

Class III

35
Q

ISKDC histologic classification of IgAV nephritis: Severe IgAV

A

Class IV or V

36
Q

Indications for renal biopsy in IgAV nephritis

A

Impaired eGFR
Severe or persistent proteinuria
Consider in the ff:
RPGN, nephrotic synd, nephritic synd

37
Q

T/F Re: arthralgia/arthritis in IgAV: Early in the disease

A

T

38
Q

T/F Re: arthralgia/arthritis in IgAV: If it precedes symptoms, usually a week or 2

A

F, a day or 2

39
Q

Arthralgia/arthritis in IgAV: MC affected joints

A

Knees and ankles

40
Q

Arthralgia/arthritis in IgAV: Erosive

A

F

41
Q

T/F Scrotal pain and swelling are relatively frequent in IgAV

A

T

42
Q

Principal lesion in IgAV nephritis

A

Endocapillary proliferative GN with increase in endothelial mesangial cells;
Deposits of principally IgA accompanied by IgG, fibrin, C3, and properdin

43
Q

T/F Absence of IgA in staining of skin lesions (histopath) excludes a diagnosis of IgAV

A

F

44
Q

Indications for GC treatment in IgAV (4)

A

Orchitis
CNS vasculitis
Pulmo hem
Severe GI

45
Q

Renal disease in IgAV, more common and severe in adults vs children

A

Adults

46
Q

Joint manifestations in IgAV, more frequent in adults vs children

A

Chidlren

47
Q

T/F NSAIDs are absolutely contraindicated in patients with IgAV and diffuse abdominal pain

A

Not contraindicated if renal fxn is normal

48
Q

Biologic agent reported to be beneficial for severe life-threatening IgAV

A

Rituximab

49
Q

SHARE guidelines for the mgt of IgAV nephritis

A

Pg 463, Fig 33.6

50
Q

Duration of IgAV in majority of patients

A

4 weeks

51
Q

Most exacerbations of IgAV take place within

A

First 6 weeks (up to 2 years from onset)

52
Q

Associated with higher recurrence rate in IgAV

A

Severe course with GI and joint involvement

53
Q

T/F Severity of LCV correlated with visceral organ involvement in IgAV

A

F

54
Q

Significant morbidity and mort in IgAV, short term

A

GI

55
Q

Significant morbidity and mort in IgAV, long term

A

renal

56
Q

Worst renal outcome in IgAV is assoc with

A

Nephritic or nephrotic synd

57
Q

Patients with IgAV clinical nephritis should be followed closely for at least how long

A

5 years

58
Q

Criteria for dx of hypersensitivity vasculitis

A

Pg 465, T33.4

59
Q

Tx for hypersensitivity vasculitis

A

Removal of precipitating agent - GC therapy if with severe cutaneous symptoms or systemic vasculitis

60
Q

IgAV vs Hypersensitivity vasculitis

A

IgAV: More freq GI, renal, palpable purpura
HV: Milder renal, freq normal ESR

Vasculitis (8 decks)

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