Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Ginjal > GN AAV > Flashcards

GN AAV Flashcards

1
Q

What is ANCA?

A

Anti Neutrophil Cytoplasmic Antibody

~ autoantibodies againts cytoplasmic antigens in neutrophils granules + monocytes lysosomes
- specifically againts MPO / PR3
- igG (in active stage)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is AAV?

A

**A group of disorder characterized by:
- destruction of small & medium sized blood vessels
- presence if circulating ANCA
**
Int Chapel Hill Concensus Conf 2012
- calssified vasculitis according to vessels size

Male predominance
Higher in 60-70 y/o
White asian

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Clinical dz of AAV

A
  1. GPA: granulomatous with polyangitis / Wegener
  2. MPA: microscopic polyangitis
  3. EGPA: Eusinophilic GPA / Churg strauss dz
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is major target antigen in AAV?

A
  1. PR3: Proteinase 3
  2. MPO: myeloperoxidase
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Higher relapse rate

A

**PR3 **ANCA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Higher mortality rate

A

MPO ANCA

why?
poor response to IS
» GS / Int fibrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Chapel Hill concensus classification
Small vessel vasculitis (svv)

A
  1. AAV
  2. Immune mediated SVV
    - anti GBM
    - ig A Nephropathy
    - anti-C1q
    - cyoglobulinaemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Chapel Hill Consensus
Medium vessels vasculitis

A
  1. PAN
  2. Kawasaki dz
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Chapel Hills Consensus
Large vessels vasculitis

Variable vessels

A

Large
1. Takayasu arteritis
2. Giant cells arteritis GCA

Variable
1. Behcet dz
2. Cogan syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

ANCA testing

A

Screening-
indirect immunoflourecence assay (IIF)
- granules disaolved post ethanol fixation
- MPO attached to perinuclear membrane – perinuclear pattern (pANCA)
-** PR3— cytoplasmic pattern (cANCA)
**
Confirmation
**Antigen specific immunoassays ** *

*2017 concensus: initial testing

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

ANCA & dz associations

A
  • PR3 ANCA - GPA (75%)
  • MPO ANCA - MPA (60%)
    - renal limted ANCA (80%)
  • Atypical ANCA (+ve IIF / -ve assays): nonvasculitic (IBD / malignancy)
  • chronic infection (IE / HIV / hep C) - either ways
  • both: drug induced vasculitis
    *
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is ANCA -ve pauci immune vasculitis

A
  • similar clinical presention
  • ANCA testing negative
  • 10% of population
  • similar response to therapy
  • — renal limited dz
  • —less severe systemic dz
  • natural inhibitor: ceruloplasmin (MPO), alpha1-antitrypsin
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Pathogenesis of AAV

A

activation of alternative pathways:
high serum / urinary C5a
low C3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is pathalogic hall mark of ANCA related GN?

A
  1. necrotizing and / or crescenteric GN
  2. without significant immune complex deposition on IF / EM
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

HPE

A

no immune complex deposit /pauci immune
a. small igG / C3
necrotizing crescenteric GN
a. necrotic area / sclerosis
b. crescent
less common
a. necrotizing extraglomerular vasculitis
b. medullary angiitis with prominent neutrophils

EGPA
+ prominent eusinophil inflammation surrounding necrotizing vasculitis or interlobar-sized and larger vessels

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

features of GPA / MPA / EGPA

A

GPA
1. constituinal symptoms
2. chronic sinusitis
3. arthalgia
4. leukocytolastic skin rash
5. AKI
6. Rbx: necrotizing crescenteric GN + pauci immune
7. serology: PR3 +ve

MPA
1. as above
2. no granulomatous manifestations
3. MPO ANCA +ve

EGPA = GPA
1. + eusinophilia
2. asthma
3. 50% MPO ANCA +ve
4. 20% renal involvement (in +ve ANCA only)

17
Q

Renal manifestation in AAV

A
  1. RPGN
  2. subnephrotic range ptnuria
  3. nephritic: hematuria + HPT
  4. Rbx findings as mentioned
18
Q
  1. prognosis
    2.prognostic factor
  2. marker to predict relapse ?
A

1.prognosis poor

  1. prognostic factor
    a.age
    b. MPO- ANCA
    c. low egfr at presentation
    d. RBx findings: if +immune complex (50%) > ptnuria > crescent / low % normal gloms / higher degree tubular atrophy
    e. freq relapse
  2. marker predict relapse
    a. ANCA level
    b. urine CD 163
    c. serum / urine CD25
    d. PR3 - more relapse
    *not a guide for tx
19
Q

Systemic involvement of AAV

A
  1. constituinal symptoms
  2. Lungs: nodules / cavitation (GPA) alveolar h/rage / fibrosis (MPA)
  3. Upper respi: rhinitis / sinusitis / otitis media / septal perforation / nasal collapse (GPA)
  4. eye / ENT: hearing loss / scleritis / uveitis / strawberry gum / palate perforation
  5. skin: leucocytoclastic vasculitis / cutaneous nodular (GPA)
  6. CNS: peripheral neuropathy / mononeuritis multiplex
  7. GI: mesenteric vasculitis
  8. heart: myocarditis / heart block
  9. thrombosis
20
Q

Dual +ve ANVA + anti GBM

A

anti GBM +ve: 10-40% +ve ANCA (MPO)
ANCA: 5-14% +ve anti GBM

severe presentation
higher morbidity / mortality

21
Q

treatment course

A

a. induction 3/6 month
b. maintenance 1-2 years

22
Q

Induction agent

A
  1. steroids
    - IV MTP 500-1g x 3/7
    - then oral pred 1mg/kg/day till 2-4 weeks – taper
  2. Cyclophosphomide
  3. Rituximab
  4. MMF
    - non life threatening
23
Q

Trials in steroid induction
MEPEX
PEXIVAS

A

*** MEPEX: **
- PLEX 7x vs high dose steroid 1g/day x 3d
-PLEX better renal recovery NOT death

* PEXIVAS trial
- PLEX vs no PLEX
- high dose vs low dose steroid
- + Cyclo / ritux
- no difference in death / ESKD in PLEX /non PLEX group & high / low dose steroid
- low dose steroid: less infection

24
Q

Trials in Cyclo induction
CYCLOPS trial

A

*** CYCLOPS trial **
- oral Cyclo (2mg/kg/day) vs IV Cyclo (15mg/kg in every 2 week) x 3-6/12
- no difference in remission rate
- oral: higher cumulative dose
- IV: lower leucopenia

25
Q

Trials in Ritux induction
RAVE
RITUXVAS

A

**1. RAVE trial **
- new & relapse AAV
- Cyclo 2mg/kg/day - Aza x 12/52 vs Ritux 375mg/m2 x 4 doses + [pred 1mg/kg/day – 40mg/d 4/52 – 0mg at week 20)
- achieved remission at 6/12 (ritux)- esc PR3
- but not superior to Cyclo
- ritux group req less stroid for

2.RITUXVAS trial

26
Q

Definition of
a. disease activity
b. remission
c. relapse
d. treatment resistant

A
  1. remission:
    - no GN / vasculitis
    - stable egfr
    - no hematuria / ptnuria
  2. relapse
    - sx of active dz after PR / CR
    - Major: life / organ threatening
    - minor
  3. tx resistant
    - persistent sx while receiving tx equal to initial IS therapy

KDIGO GN 2021

Ginjal (14 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions