P219
A rationale for the wider use of plasma exchange in the management of ANCA vasculitis
Andrew Saunders, Neeraj Dhaun, David C Kluth. Royal Infirmary of Edinburgh
Introduction
Systemic vasculitis associated with autoantibodies to neutrophil cytoplasmic antigens (ANCA) is
the most frequent cause of rapidly progressive glomerulonephritis. Data suggest a role for plasma
exchange (PEX) on top of standard immunosuppression in improving disease outcomes. We aimed
to define a rationale for which patients presenting with ANCA disease may benefit from PEX.
Methods
Patients presenting with ANCA vasculitis between September 2006 and April 2013 were
prospectively allocated to treatment with PEX (8 centrifugal exchanges) alongside standard of care
if they fulfilled any of the following criteria at presentation: 1. serum creatinine >500µmol/l or
dialysis-requiring renal failure, 2. alveolar haemorrhage, 3. renal disease with ≥30% focal and
necrotising lesions ± cellular crescents on renal biopsy. Patients were followed up for the time
period of the study with study endpoints including disease remission and relapse, cumulative
immunosuppressive burden at 3 months, and all-cause mortality.
Results
104 patients presented with a new diagnosis of ANCA vasculitis within the study period.
Comparisons between the two groups are shown below (mean ± SD, ns, not significant).
Characteristic
Age
Male /female
ANCA status: PR3+ vs. MPO+ vs. negative
Renal involvement
Lung involvement
At presentation
Creatinine (µmol/l)
CRP (mg/l)
Haemoglobin (g/l)
Dialysis-requiring
Induction period (~3 months)
Number receiving Glucocorticoids
Cyclophosphamide
MMF
Rituximab
Cumulative glucocorticoid dose (g)
Cumulative cyclophosphamide dose (g)
Number entering remission
Time to remission (days)
At 12 months
Creatinine (µmol/l)
Dialysis-requiring
Number of patients with disease relapses (%)
Number of patients died (%)
No PEX (n=46)
61 ± 15
26/20
26 vs. 15 vs. 4
35
26
140 ± 90
48 ± 61
116 ± 21
1
PEX (n=58)
60 ± 17
27/31
25 vs. 30 vs. 3
57
30
370 ± 259
105 ± 82
94 ± 18
20
p value
ns
ns
ns
ns
p = 0.000
p = 0.001
p = 0.000
p = 0.000
46
23
8
10
2.5 ± 0.4
8.0 ± 3.6
44
118 ± 200
120 ± 46
0
12 (26)
5 (11)
58
45
16
7
2.3 ± 0.2
5.4 ± 3.0
57
83 ± 39
191 ± 172
5
8 (14)
3 (5)
ns
p = 0.005
ns
ns
p = 0.000
p = 0.002
ns
ns
p = 0.02
p = 0.04
ns
ns
Conclusion
Despite more severe disease at presentation, those patients receiving PEX had similar disease
outcomes to those not receiving it. PEX treatment was associated with a lower cumulative dose of
glucocorticoid and cyclophosphamide. Extent of histological injury should be considered alongside
current suggested criteria for treatment of patients with ANCA vasculitis with PEX.