Muthia Faurin

Allergy Immunology Subdivision of Internal Medicine

Medical Faculty of Andalas University/M.Djamil Hospital
 01
INTRODUCTIO
N
ANCA – AAV
A group of disorders including GPA, EGPA and MPA
Characteristic | systemic necrotizing inflammation in small- and medium- size
vessels that leading to end-organ damage

● The overall annual incidence of AAV is estimated to be around 0.4-24 per

million population per year
● The prevalence of AAV is more common in European ancestry ; MPA in
Southern Europe and Asia ; GPA in Northern Europe

The incidence increases with age (a peak age around 60-70 years old)

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 IMMUNOSUPRESSION AGENTS
Indication | to induce remission and as a long-term maintenance treatment

Cyclophosphamide or Rituximab
Plasmapheresis (PLEX)
with Glucocorticoid

as an induction agents for rapidly progressive renal failure or

severe diffuse pulmonary haemorrhage

Azathioprine, Rituximab, or Methotrexate with Glucocorticoid tapering

as an maintenance therapy

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 RENAL INVOLVEMENT
“one of the main predictors of mortality and morbidity in AAV”

1. Elevated serum creatinine at the time of diagnosis

2. Renal histopathological (necrotizing and crescentic glomerulonephritis without
significant immune complex deposition) --- GOLD STANDARD !!!

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 02
METHODS
RETROSPECTIVE COHORT
STUDY
Nephrology department in Beaumont Hospital, Dublin,
between the 1st of January 2012 and the 31st of December 2017,
with follow-up period until the 31st of December 2019

Electronic renal database (eMED)

Data Source (data on outpatient encounters, inpatient care, patient laboratory & histology results)

Sample Criteria Inclusion Criteria

1. Clinical diagnosis of AAV consistent with the Chapel Hill consensus,

positive serology (anti-PR3 or antiMPO antibody positivity), renal biopsy
(pauci-immune necrotising glomerulonephritis), or active urine sediment
(glomerular haematuria or red cell casts & proteinuria)
2. Clinical features consistent with AAV
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Sample Criteria Exclusion

1. Diagnosis of vasculitis other than granulomatosis with polyangiitis or

microscopic polyangiitis
2. Renal histological features suggestive of other type of
glomerulonephritis
3. Age less than 16 years
4. Incomplete records

Patient Age, gender, comorbidities, BVAS and organ systems involved,

Characteristics creatinine levels, anti-PRA3/anti-MPO titres

Outcomes ESRD = requiring renal replacement therapy

CKD = eGFR of less than 60 ml/min/1.73m2)
Preservation of renal function= eGFR of more than 60
ml/min/1.73m2)
Death

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 04
RESULTS
 42
PATIENTS
PR3-ANCA- & MPO-ANCA-
associated vasculitis
were identified between 2012 and 2017, with a
median follow-up period of 6 years

6 patients
were excluded

36
PATIENTS

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 42
PATIENTS
PR3-ANCA- & MPO-ANCA-
associated vasculitis
were identified between 2012 and 2017, with a
median follow-up period of 6 years

6 patients
were excluded

36
PATIENTS

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 05
OUTCOMES
 RENAL BIOPSY PERFORMED
32
PATIENTS

● All the performed biopsies were assessed with light microscopy and
immunofluorescence
● Renal biopsy showed pauci-immune necrotising and crescentic
glomerulonephritis

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INDUCTION & MAINTENANCE
THERAPY
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7 PATIENTS 4 PATIENT
PATIENTS
Cyclophosphamide Rituximab Azathioprine
for induction as an induction

1 PATIENT
Combination of low-dose
Cyclophosphamide and
Rituximab

“the majority of patients were on Azathioprine (61.1%) as maintenance therapy”

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 RELAPSE

7 PATIENTS
“mean time to relapse was 3.4 years”

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 OUTCOME
Progression to ESRD or Death

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4 PATIENT 6 PATIENT
PATIENTS PATIENTS
ESRD Death CKD Preservation of
renal function

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 OUTCOME
11 15
4 PATIENT 6 PATIENT
PATIENTS PATIENTS
ESRD Death CKD Preservation of
renal function

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 06
DISCUSSION
 Cyclophosphamide
the choice of induction therapy in AAV with severe renal involvement

30.6% patients developed ESRD 11.1% patients death

In this cohort,
the median creatinine at presentation was 301 μmol/L (3.4 mg/ dL)  high
incidence of ESRD within the first 13 months of diagnosis

✓ similar to a large Dutch study

MPA was relatively more common in our study compared to GPA

✓ similar to UKIVAS

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 Azathioprine Rituximab
the choice of maintenance therapy the choice of maintenance remission in AAV

PLEX
for severe renal involvement with or without pulmonary haemorrhage
(serum creatinine more than 500 µmol/L)

In this cohort,
Rituximab was favoured as an induction agent in the relapse of AAV (86%)

19% of patients relapsed, with a median time to relapse being three year

• highest risk of relapse is seen in AAV with anti-PR3 and preserved renal function
• ANCA positivity at the time of completion of induction therapy
• staphylococcus aureus nasal carriage
• choice of induction and maintenance therapy
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 06
CONCLUSIONS
CONCLUSIO
NS
● Cyclophosphamide is used more commonly as an inducing
immunosuppression therapy
● Rituximab is utilised more often in relapsing disease of AAV in
this single-centre experience
● Despite aggressive immunosuppression therapy, the incidence of
ESRD and death remains high in these patients

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