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Vasculitis > Ch. 36 AAV > Flashcards

Ch. 36 AAV Flashcards

1
Q

AAVs

A

(4) GPA, MPA, EGPA, Renal-limited pauci-immune GN

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2
Q

AAVs and associated ANCAs

A

EGPA: pANCA > cANCA
cANCA (PR3-ANCA): GPA>MPA>EGPA
pANCA (MPO-ANCA): MPA>GPA

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3
Q

Heterogenous group of antibodies that bind to antigens in the primary granules of neutrophils and lysosomes of monocytes

A

ANCAs

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4
Q

ANCAs are present in what vascuitides

A

AAVs
Renal-limited vasculitis
Certain drug-induced vasculitis syndromes

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5
Q

GPA triad

A

Upper respiratory tract inflamm
Lower respiratory tract inflamm
Renal disease

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6
Q

Genes associated with GPA

A

MHC Class II HLA-DP region

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7
Q

Environmental factors identified as triggers for GPA

A

Infections (S. aureus, other respi tract infections)
Drugs (PTU, hydralazine, minocycline)
Silica dust

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8
Q

Key effector cells of endothelial injury in GPA

A

Primed neutrophils activated by ANCA

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9
Q

Other players in the inflammatory loop of GPA besides primed neutrophils

A

Alternative complement pathways, autoantibodies, cellular immunity

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10
Q

MC features of GPA at onset

A

Renal > pulmonary > ENT

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11
Q

Limited or localized GPA is defined by

A

Absence of renal disease

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12
Q

ACR criteria for GPA

A

2/4
1. Nasal/oral inflammation
2. Abnormal cxr
3. Abnormal ua
4. Granulomatous inflammation

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13
Q

EULAR/PRINTO/PRES criteria for GPA

A

3/6
1. Upper airway involvement
2. LTB stenosis
3. Pulmonary involvement
4. Renal involvement
5. Granulomatous inflamm
6. ANCA positivity

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14
Q

Classic presentation of GPA

A

Pulmo hem + GN (pulmo-renal syndrome)

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15
Q

Condition wherein tissue diagnosis is desirable in GPA

A

Single organ involvement

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16
Q

AAV where there is pauci-immune GN and characteristic serology (elevated ANCA)

A

GPA

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17
Q

GPA vs MPA

A

GPA: Upper airway disease

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18
Q

MC clinical manifestations in children with GPA

A

Constitutional > Renal > Pulmonary

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19
Q

T/F Upper respi tract biopsy in patients with GPA have very high yield

A

F, disappointingly low

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20
Q

T/F Standard inflamm markers are helpful in determining level of disease activity in GPA

A

F

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21
Q

Useful markers to track inflammation in GPA

A

S100A8/A9
S100A12
Neutrophil count

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22
Q

Pathologic characteristic of GPA

A

Granulomata

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23
Q

T/F Presence of eosinophils in small numbers indicate EGPA instead of GPA

A

F, small numbers of eosinophils may be seen in GPA and EGPA is characterized by large numbers of eos

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24
Q

MC reported renal lesion of GPA

A

Renal granulomata are RARE; Extracapillary proliferation and crescent formation in a focal and segmental pattern; IF shows pauci-immune pattern

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25
T/F GPA is usually monophasic
F, follow a chronic relapsing course
26
Usual duration of treatment of GPA
3-6m induction, 2-4y maintenance
27
Cornerstone of therapy for remission induction and maintenance in GPA
Corticosteroids
28
Induction therapy for GPA
MILD: Prednisone 1-2mkday or SEVERE: MPPT 30mkdose for 3 days IV PLUS CYC or MTX
29
Target dose for corticosteroid used in GPA induction
0.2mkday or 10mg/day whichever is lower by 6th month (induction) to be continued until 12 months (maintenance phase)
30
T/F For GPA patients who do not respond well to IV CYC, patient may be shifted to oral CYC
T
31
Indications for RTX as induction in GPA
1. Failed CYC 2. Relapsing disease 3. CYC toxicity
32
Options for maintenance therapy for GPA
Pred + AZA or MTX or RTX for minimum of 18m to 24m
33
Standard of care for remission maintenance in adults with AAV
AZA
34
Alternative to AZA for remission maintenance in AAV
MTX
35
Option for refractory GPA
RTX
36
For pedia GPA, there is an increasingly high rate of ___ involvement accumulating with time
ENT
37
AAV originally described as a subset of PAN
MPA
38
AAV: Necrotizing GN
MPA
39
Necrotizing vasculitis with few or no immune deposits, predominantly affecting small vessels
MPA
40
GPA vs MPA: No strong assoc with infection
MPA
41
MC pulmo manifestation of MPA
Pulmo hem
42
Predominant skin presentation in MPA
PUrpura
43
GPA vs MPA: Pulmo manif are more common
GPA
44
MC clinical features of MPA
Same with GPA: Constitutional > renal > pulmo
45
MC constitutional symptoms for both GPA and MPA
Malaise/fatigue > fever > wt loss
46
MC renal manif for both GPA and MPA
Biopsy-proven nephritis
47
MC msk manif for both GPA and MPA
Arthralgia/arthritis
48
The only ENT manif reported for MPA
Oral ulcer
49
MC CNS manif for both GPA and MPA
severe headache
50
MC GI manif for both GPA and MPA
Nonspecific abdominal pain
51
MC cutaneous manif for both GPA and MPA
Palpable purpura
52
GPA vs MPA: Pulmonary capillaritis
MPA
53
GPA vs MPA: Pulmo nodules
GPA
54
MPA vs anti-GBM disease
Anti-GBM: IF showing linear deposition of IgG along GBM
55
GPA vs MPA
GPA: Granulomatous inflamm URT involvement partic nasal septal perforation and/or saddle nose deformity Nodules instead of capillaritis
56
T/F Anti-GBM disease may also present with ANCA
T
57
Treatment for MPA
Similar to other AAVs: CS + CYC, RTX
58
EGPA vasculitis commonly involves what organ systems
CV CNS GI
59
Eosinophil-rich granulomatous inflamm of the respi tract
EGPA
60
Main ANCA seen in EGPA
MPO ANCA
61
ACR EGPA criteria
At least 4 of the ff: Asthma Eosinophilia (>10% of diff count) History of allergy Mono- or polyneuropathy Pulmonary infiltrates Paranasal sinus abn Extravascular eosinophils
62
3 phases of EGPA that DO NOT usually occur sequentially
1) Asthma and other allergic manifestations 2) Eosinophilic phase: Eosinophilia + Pulmo infiltrates 3) Vasculitis
63
MC presenting symptom of EGPA
Asthma > nonfixed pulmo infiltrates > sinusitis > skin
64
Hallmark of EGPA
Asthma
65
T/F Asthma of EGPA usually requires CS for treatment
T
66
T/F Renal disease of EGPA is usually mild and rarely progresses
T
67
T/F HRCT of lung is recommended if suspicion of EGPA is high
T, CXR may be normal in the presence of lung disease
68
Treatment for EGPA
Like other AAVs (GPA and MPA)
69
T/F EGPA usually has a prolonged prodromal course of many years
T

Vasculitis (8 decks)

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