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Page 6 of 14 Skopis et al. Vessel Plus 2020;4:30 I http://dx.doi.org/10.20517/2574-1209.2020.42

Rituximab Retrospective study of the To investigate the Rituximab was well Half of the patients
maintenance therapy outcomes and tolerance efficacy of rituximab tolerated and maintained had received additional
for granulomatosis of patients with MPA as a maintenance remission of patients with immunosuppression
with polyangiitis and and GPA treated with therapy in patients with GPA and MPA however, it concomitantly with
microscopic polyangiitis, rituximab maintenance AAV who achieved did not completely prevent rituximab, making it
2012 [22] therapy disease remission relapses difficult to discern if the
with conventional **The preliminary results low relapse rates were
immunosuppressants or of this study were to solely attributable to
rituximab be confirmed by the rituximab use
MAINRITSAN trial which Preliminary study which
was in progress when the did not allow conclusions
results of this study were about the exact role of
published rituximab in treating AAV
MAINRITSAN trial, Nonblinded, randomized To compare rituximab Rituximab infusions of Trial was not blinded
2014 [23] controlled trial infusions to azathioprine 500 mg given every 6 Fewer patients with anti-
as maintenance therapy months were superior MPO ANCA positive
in patients with ANCA to azathioprine as vasculitis, MPA, or renal
associated vasculitis maintenance therapy limited disease
(MPA, GPA, and renal- in AAV, particularly in
associated ANCA patients who are PR-3
vasculitis) ANCA positive
MAINRITSAN2 trial, Open-label, pragmatic, To compare rituximab There was not a significant Open-labeled but all
2018 [24] multicenter randomized infusions tailored to the difference in the number relapses were assessed
controlled trial appearance of ANCA of relapses in the tailored by an independent
autoantibodies, increasing infusion group vs. the Adjudication Committee
ANCA titers and/or the fixed dose regimen group; who was not aware of
presence of CD19+ B tailored infusion group the treatment arm or the
cells in the circulation received fewer infusions circulating CD19+ B cell
measured every three while still maintaining a count
months to fixed dose low relapse rate There were 59 centers
regimens of rituximab in with testing performed
patients with GPA or MPA at each individual center
as maintenance therapy (as opposed to all testing
being done in the same
laboratory), however, all
labs for a given patient
had to be drawn at the
same laboratory
RITAZAREM trial, 2019 [25] International, multi-center, To compare the efficacy Rituximab was superior Investigators were given
open-labeled, randomized, of rituximab vs. oral to azathioprine as the option to choose the
controlled trial azathioprine as a maintenance therapy glucocorticoid tapering
maintenance therapy in in AAV patients with a regimen after induction
patients with a history of history of prior relapses therapy as opposed to
relapsing AAV who had using a blinded, randomly
received induction therapy assigned tapering
with rituximab schedule
Prolonged B cell Retrospective case series To investigate the Prolonged B cell depletion Small patient cohort (5
depletion with rituximab efficacy of rituximab following rituximab patients) makes results
is effective in treating infusions in treating infusion was effective in hard to generalize
refractory pulmonary pulmonary granulomas reducing both the size
granulomatous in patients with GPA and number of pulmonary
inflammation in who were previously nodules in these patients
granulomatosis with resistant to traditional for at least 18 months
polyangiitis (GPA), immunosuppressive after treatment
2014 [26] treatment
Rituximab for treatment Retrospective multi-center To investigate the There was no difference Concomitant
of severe renal disease study efficacy of rituximab and in outcomes between glucocorticoid
in ANCA associated glucocorticoids alone vs. the rituximab and administration with
vasculitis, 2016 [28] rituximab, glucocorticoids glucocorticoids arm vs. rituximab makes it
and cyclophosphamide rituximab, glucocorticoids difficult to discern if the
as a treatment for AAV and cyclophosphamide results can be solely
patients with severe renal arm attributable to rituximab
disease
Rituximab in the Retrospective analysis of To evaluate the efficacy Four weeks after Small patient cohort (8
treatment of refractory interventional case series of rituximab in patients treatment with rituximab, patients) makes results
scleritis in patients with with a history of GPA all patients showed hard to generalize
polyangiitis (Wegener’s), who developed scleritis improvement of refractory
2015 [29] that was refractory necrotizing anterior
to conventional scleritis and no further
immunosuppressant disease progression
therapy