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Skopis et al. Vessel Plus 2020;4:30  I  http://dx.doi.org/10.20517/2574-1209.2020.42                                                 Page 5 of 14

               Table 1. Studies regarding maintenance therapy with Rituximab in ANCA-associated vasculitis
                Study and year of study  Study design  Objective of study   Results        Study limitations
                Response of Wegner’s   Case Report   To describe the successful  Rituximab was able   The report only describes
                granulomatosis to                   use of rituximab to   to successfully induce   the response of one
                Anti-CD20 chimeric                  treat a patient with   and maintain disease   individual to rituximab
                monoclonal antibody                 chronic, relapsing GPA   remission in a patient   which fails to generalize
                therapy, 2001 [16]                  who did not tolerate   with chronic, relapsing   the results to other
                                                    cyclophosphamide   GPA resistant to other   patients with ANCA
                                                    therapy and was   immunosuppressants   associated vasculitis
                                                    resistant to treatment
                                                    with glucocorticoids,
                                                    azathioprine and
                                                    mycophenolate mofetil
                Nine patients with anti-  Case series which   To review the outcomes   Rituximab was efficient   No control arm
                neutrophil cytoplasmic   included with structured  of 9 patients with MPA   and safe as an induction   Possible selection bias
                antibody-associated   patient follow up  and GPA treated with   and maintenance therapy  Patients received
                vasculitis successfully             rituximab who were   for patients with MPA and  additional
                treated with rituximab,             either resistant to or   GPA        immunosuppressive
                2005 [17]                           had recurrent relapses              medications while they
                                                    after cessation of                  were on treatment with
                                                    cyclophosphamide                    rituximab
                Adjunction of rituximab   Retrospective study   To investigate rituximab   Treatment of relapsing/  Patients were
                to steroids and   of 8 patients with   use in conjunction with   refractory GPA with   receiving concomitant
                immunosuppressants   refractory or relapsing   ongoing steroid and   rituximab in conjunction   immunosuppressive
                for refractory/relapsing   GPA who received   immunosuppressant   with steroids/  therapy so it is difficult
                Wegner’s granulomatosis: rituximab infusions in   therapy as a treatment   immunosuppressants   to tell if results could be
                a study on 8 patients,   addition to their ongoing  for relapsing/refractory   resulted in good clinical   attributed to rituximab
                2007 [18]         immunosuppressive   GPA and to determine the  outcomes   alone
                                  therapy           frequency of infusions,   There was a dissociation
                                                    time to patient response   in the time to response of
                                                    and, effects on the various  vasculitis manifestations
                                                    manifestations of GPA  (improved over days to
                                                                      weeks) vs. granulomatous
                                                                      manifestations (improved
                                                                      over several months) to
                                                                      rituximab
                A multicenter survey of   Standardized,   To determine if rituximab  Rituximab was found to be  Possibility of positive
                Rituximab therapy for   retrospective data   is a safe and effective   successful as an induction  outcome bias given
                refractory antineutrophil  collection from 65 patients option in treating patients  therapy in patients with   that the study was a
                cytoplasmic antibody-  at 4 centers in the UK with with ANCA associated   AAV  retrospective review
                associated vasculitis,   a history of refractory AAV vasculitis  Additionally, patients
                2009 [19]         who received rituximab as           who received preemptive
                                  induction therapy (largest          retreatment in the absence
                                  series reported at that             of any signs of a relapse
                                  time)                               with a regimen of 1 g
                                                                      rituximab every 6 months
                                                                      had no disease relapse
                                                                      at eleven- month follow
                                                                      up, suggesting rituximab
                                                                      as a viable maintenance
                                                                      therapy
                Rituximab as maintenance Retrospective review of   To determine the efficacy  Rituximab was safe and   Comparison with other
                therapy for anti-  39 patients with AAV who  and safety of rituximab   effective in maintaining   studies is limited because
                neutrophil cytoplasmic   received maintenance   infusions as maintenance  disease remission in   this cohort had lower
                antibody-associated   therapy with rituximab  therapy in patients   patients with ANCA-  disease activity at study
                vasculitis, 2010 [20]               with ANCA-associated   associated vasculitis   onset
                                                    vasculitis who had
                                                    achieved complete or
                                                    partial remission
                Rituximab for remission   Single-center historical   To determine the efficacy  Rituximab was effective   Open-label administration
                induction and     cohort study observing   of rituximab as a therapy  and well tolerated   of rituximab
                maintenance in refractory  all patients (53 total)   for maintenance of   as an induction and   Experience of study was
                granulomatosis with   with a history of chronic   remission in patients   maintenance therapy in   only from one center
                polyangiitis (Wegner’s),   relapsing GPA treated   with a history of chronic   patients with a history of   with a predominantly
                2012: ten year experience  with rituximab therapy   relapsing refractory GPA  chronic relapsing GPA   Caucasian population
                at a single center [21]  from January 1, 2000 to                        of Scandinavian and
                                  May 31, 2010                                          Northern European
                                                                                        background
                                                                                        Except for 1 patient, all
                                                                                        patients were PR-3 ANCA
                                                                                        positive
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