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

Question 1

Leukocytoclasis is the predominant inflammatory reaction in which vasculitides

Answer 1

1) IgAV
2) Hypersensitivity vasculitis
3) Mixed cryoglobulinemia

Question 2

Conditions associated with leukocytoclastic vasculitis

Answer 2

Box 33.1 (p.457)

Question 3

MC systemic vasculitis of childhood

Answer 3

IgAV

Question 4

Diagnostic triad of IgAV

Answer 4

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

Question 5

2010 Classification Criteria for IgAV

Answer 5

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)

Question 6

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

Answer 6

T

Question 7

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

Answer 7

T

Question 8

IgAV most frequently occurs in what age group

Answer 8

3-12y

Question 9

IgAV is rare in what age

Answer 9

<2y

Question 10

IgAV is more common in what gender

Answer 10

M

Question 11

T/F IgAV is often preceded by an URTI

Answer 11

T, 30-50%

Question 12

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

Answer 12

T

Question 13

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

Answer 13

F

Question 14

Suggested pathogenesis of IgAV nephritis

Answer 14

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

Question 15

Most specific and sensitive criterion for IgAV

Answer 15

Histopath

Question 16

Constitutional signs often present in IgAV

Answer 16

Low-grade fever or malaise

Question 17

Presenting symptom in ~3/4 of patients with IgAV

Answer 17

Skin manifestations

Question 18

T/F Ulcerations do not develop in IgAV

Answer 18

F, may occasionally develop in large ecchymotic areas

Question 19

GI symptoms of IgAV usually appears ___

Answer 19

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

Question 20

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

Answer 20

T

Question 21

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

Answer 21

Small bowel

Question 22

MC surgical complication of IgAV

Answer 22

Intussusception

Question 23

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

Answer 23

1/3, <10%

Question 24

T/F Renal disease in IgAV commonly precedes purpura

Answer 24

F, seldom

Question 25

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

Answer 25

4-6 weeks of rash onset

Question 26

risk factors for renal involvement in IgAV

Answer 26

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

Question 27

Definitions of severity of IgAV nephritis (SHARE): Mild

Answer 27

Normal GFR and mild/moderate proteinuria

Question 28

Definitions of severity of IgAV nephritis (SHARE): Moderate

Answer 28

<50% crescents Impaired GFR OR severe presistent proteinuria

Question 29

Definitions of severity of IgAV nephritis (SHARE): Severe

Answer 29

>50% crescents Impaired GFR OR presistent proteinuria

Question 30

PErsistent proteinuria (UPCr)

Answer 30

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

Question 31

Mild proteinuria (UPCr)

Answer 31

<100mg/mmol

Question 31

Moderate proteinuria (UPCr)

Answer 31

100-250mg/mmol

Question 32

Severe proteinuria (UPCr)

Answer 32

>250mg/mmol

Question 33

ISKDC histologic classification of IgAV nephritis: Mild IgAV

Answer 33

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

Question 34

ISKDC histologic classification of IgAV nephritis: Moderate IgAV

Answer 34

Class III

Question 35

ISKDC histologic classification of IgAV nephritis: Severe IgAV

Answer 35

Class IV or V

Question 36

Indications for renal biopsy in IgAV nephritis

Answer 36

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

Question 37

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

Answer 37

T

Question 38

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

Answer 38

F, a day or 2

Question 39

Arthralgia/arthritis in IgAV: MC affected joints

Answer 39

Knees and ankles

Question 40

Arthralgia/arthritis in IgAV: Erosive

Answer 40

F

Question 41

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

Answer 41

T

Question 42

Principal lesion in IgAV nephritis

Answer 42

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

Question 43

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

Answer 43

F

Question 44

Indications for GC treatment in IgAV (4)

Answer 44

Orchitis CNS vasculitis Pulmo hem Severe GI

Question 45

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

Answer 45

Adults

Question 46

Joint manifestations in IgAV, more frequent in adults vs children

Answer 46

Chidlren

Question 47

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

Answer 47

Not contraindicated if renal fxn is normal

Question 48

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

Answer 48

Rituximab

Question 49

SHARE guidelines for the mgt of IgAV nephritis

Answer 49

Pg 463, Fig 33.6

Question 50

Duration of IgAV in majority of patients

Answer 50

4 weeks

Question 51

Most exacerbations of IgAV take place within

Answer 51

First 6 weeks (up to 2 years from onset)

Question 52

Associated with higher recurrence rate in IgAV

Answer 52

Severe course with GI and joint involvement

Question 53

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

Answer 53

F

Question 54

Significant morbidity and mort in IgAV, short term

Answer 54

GI

Question 55

Significant morbidity and mort in IgAV, long term

Answer 55

renal

Question 56

Worst renal outcome in IgAV is assoc with

Answer 56

Nephritic or nephrotic synd

Question 57

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

Answer 57

5 years

Question 58

Criteria for dx of hypersensitivity vasculitis

Answer 58

Pg 465, T33.4

Question 59

Tx for hypersensitivity vasculitis

Answer 59

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

Question 60

IgAV vs Hypersensitivity vasculitis

Answer 60

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