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Abstract Number: 2634

ANCA Response upon Rituximab or Cyclophosphamide in ANCA-associated Vasculitis Patients

Laura van Dam1, Ebru Dirikgil 2, Edwin Bredewold 2, Argho Ray 2, Ton Rabelink 2, Cees van Kooten 2 and Onno Teng 2, 1LUMC, Leiden, Zuid-Holland, Netherlands, 2LUMC, Leiden, Netherlands

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: ANCA, Rituximab, systemic vasculitides and biologic drugs, Vasculitis

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Session Information

Date: Tuesday, November 12, 2019

Title: Vasculitis – ANCA-Associated Poster I

Session Type: Poster Session (Tuesday)

Session Time: 9:00AM-11:00AM

Background/Purpose: Recent studies have demonstrated that in patients with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) with successful remission-induction (RI) after cyclophosphamide (CYC), maintenance treatment with ANCA-guided rituximab (RTX) improved relapse free survival (RFS). However, current recommended RI treatment is RTX, CYC or a combination of both. As yet it is unclear how these different RI therapies affect ANCA levels. Therefore, this study aimed to investigate the potential of RTX, CYC, or RTX+CYC to achieve an ANCA-negative status and its effect on RFS to further improve the insight on ANCA-guided treatment in AAV patients.

Methods: This retrospective single centre study involved 129 ANCA-positive AAV patients treated with 200 remission-induction regimens, including RTX (n=109), CYC (n=66), or RTX+CYC (n=25) between 1990 – 2018 with a mean follow-up (FU) of 328 weeks. ANCA serum levels and major RFS were assessed.

Results: Within 6 months, 23% of RTX-treated, 50% of CYC-treated and 40% of RTX+CYC-treated AAV patients achieved an ANCA-negative status (p=0.0001) (Figure 1A). Time to ANCA negativity was significantly shorter after CYC+/- RTX (mean±SD: 11±6 weeks) as compared to RTX (16±6 weeks; p=0.02). ANCA reappearance within 1 year after achieving ANCA negativity, occurred in 9 out of 38 (24%) RTX-treated, 17 out of 44 (39%) CYC-treated and 2 out of 14 (14%) RTX+CYC-treated patients (p=0.17), which happened significantly faster in CYC-treated patients at an average of 18 weeks as compared to RTX+/- CYC at an average of 30 weeks (p=0.003) (Figure 1B). Both 1yr and 2yr major RFS was significantly less for RTX-treated (86% and 68%) as compared to CYC-treated (97% and 91%) and RTX+CYC-treated patients (100%, 91%) (p=0.02, p=0.005). Overall, patients that reached an ANCA-negative status had a better 2yr-RFS. ANCA reappearance associated with major relapses in RTX-treated group (56% vs 7%; p=0.002) (Figure 1C) but not in CYC-treated group (12% vs 7%; p=0.68) (Figure 1D).

Conclusion: This study demonstrates that an ANCA-negative status was achieved more frequently and quicker with CYC +/- RTX as compared to RTX and associated with a better 2yr-RFS. ANCA reappearance was associated with relapses in RTX-treated patients but not in CYC-treated patients. Thus, monitoring ANCAs to guide tailored maintenance treatment is most relevant in RTX-treated AAV patients.

Figure 1. ANCA responses are assessed in ANCA-associated vasculitis patients after different remission-induction regimens. A- The achievement of ANCA negativity within 6 months after remission induction -RI–therapy with Rituximab -blue line-, Cyclophosphamide -green line- or a combination of Rituximab plus Cyclophosphamide -black line-. B- ANCA reappearance within one year after achievement of ANCA negativity was investigated for the different RI regimens. C- 2-year major relapse free survival in C- RTX-treated patients and D- CYC-treated patients with -red line- or without ANCA reappearance -black line-.


Disclosure: L. van Dam, None; E. Dirikgil, None; E. Bredewold, None; A. Ray, None; T. Rabelink, None; C. van Kooten, None; O. Teng, None.

To cite this abstract in AMA style:

van Dam L, Dirikgil E, Bredewold E, Ray A, Rabelink T, van Kooten C, Teng O. ANCA Response upon Rituximab or Cyclophosphamide in ANCA-associated Vasculitis Patients [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/anca-response-upon-rituximab-or-cyclophosphamide-in-anca-associated-vasculitis-patients/. Accessed .
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