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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
Q

T/F GPA is usually monophasic

A

F, follow a chronic relapsing course

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26
Q

Usual duration of treatment of GPA

A

3-6m induction, 2-4y maintenance

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27
Q

Cornerstone of therapy for remission induction and maintenance in GPA

A

Corticosteroids

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28
Q

Induction therapy for GPA

A

MILD: Prednisone 1-2mkday or SEVERE: MPPT 30mkdose for 3 days IV
PLUS
CYC or MTX

29
Q

Target dose for corticosteroid used in GPA induction

A

0.2mkday or 10mg/day whichever is lower by 6th month (induction) to be continued until 12 months (maintenance phase)

30
Q

T/F For GPA patients who do not respond well to IV CYC, patient may be shifted to oral CYC

31
Q

Indications for RTX as induction in GPA

A
  1. Failed CYC
  2. Relapsing disease
  3. CYC toxicity
32
Q

Options for maintenance therapy for GPA

A

Pred +
AZA or MTX or RTX
for minimum of 18m to 24m

33
Q

Standard of care for remission maintenance in adults with AAV

34
Q

Alternative to AZA for remission maintenance in AAV

35
Q

Option for refractory GPA

36
Q

For pedia GPA, there is an increasingly high rate of ___ involvement accumulating with time

37
Q

AAV originally described as a subset of PAN

38
Q

AAV: Necrotizing GN

39
Q

Necrotizing vasculitis with few or no immune deposits, predominantly affecting small vessels

40
Q

GPA vs MPA: No strong assoc with infection

41
Q

MC pulmo manifestation of MPA

42
Q

Predominant skin presentation in MPA

43
Q

GPA vs MPA: Pulmo manif are more common

44
Q

MC clinical features of MPA

A

Same with GPA:
Constitutional > renal > pulmo

45
Q

MC constitutional symptoms for both GPA and MPA

A

Malaise/fatigue > fever > wt loss

46
Q

MC renal manif for both GPA and MPA

A

Biopsy-proven nephritis

47
Q

MC msk manif for both GPA and MPA

A

Arthralgia/arthritis

48
Q

The only ENT manif reported for MPA

A

Oral ulcer

49
Q

MC CNS manif for both GPA and MPA

A

severe headache

50
Q

MC GI manif for both GPA and MPA

A

Nonspecific abdominal pain

51
Q

MC cutaneous manif for both GPA and MPA

A

Palpable purpura

52
Q

GPA vs MPA: Pulmonary capillaritis

53
Q

GPA vs MPA: Pulmo nodules

54
Q

MPA vs anti-GBM disease

A

Anti-GBM: IF showing linear deposition of IgG along GBM

55
Q

GPA vs MPA

A

GPA:
Granulomatous inflamm
URT involvement partic nasal septal perforation and/or saddle nose deformity
Nodules instead of capillaritis

56
Q

T/F Anti-GBM disease may also present with ANCA

57
Q

Treatment for MPA

A

Similar to other AAVs: CS + CYC, RTX

58
Q

EGPA vasculitis commonly involves what organ systems

59
Q

Eosinophil-rich granulomatous inflamm of the respi tract

60
Q

Main ANCA seen in EGPA

61
Q

ACR EGPA criteria

A

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
Q

3 phases of EGPA that DO NOT usually occur sequentially

A

1) Asthma and other allergic manifestations
2) Eosinophilic phase: Eosinophilia + Pulmo infiltrates
3) Vasculitis

63
Q

MC presenting symptom of EGPA

A

Asthma > nonfixed pulmo infiltrates > sinusitis > skin

64
Q

Hallmark of EGPA

65
Q

T/F Asthma of EGPA usually requires CS for treatment

66
Q

T/F Renal disease of EGPA is usually mild and rarely progresses

67
Q

T/F HRCT of lung is recommended if suspicion of EGPA is high

A

T, CXR may be normal in the presence of lung disease

68
Q

Treatment for EGPA

A

Like other AAVs (GPA and MPA)

69
Q

T/F EGPA usually has a prolonged prodromal course of many years

Vasculitis (8 decks)

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