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Abstract: SA-PO661

ANCA Response on Rituximab or Cyclophosphamide in ANCA-Associated Vasculitis Patients

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • van Dam, Laura Sophie, LUMC, Leiden, Netherlands
  • Dirikgil, Ebru, LUMC, Leiden, Netherlands
  • Bredewold, Edwin, LUMC, Leiden, Netherlands
  • Rabelink, Ton J., LUMC, Leiden, Netherlands
  • van Kooten, Cees, LUMC, Leiden, Netherlands
  • Teng, Yoe Kie Onno, LUMC, Leiden, Netherlands

Group or Team Name

  • Lupus, Vasculitis and Complement Center of Expertise (LuVaCS) LUMC Leiden
Background

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 therapy is RTX, CYC or the combination. 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 cohort 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 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). 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 31 (29%) RTX-treated, 17 out of 43 (39%) CYC-treated and 2 out of 12 (17%) RTX+CYC-treated patients (p=0.2), 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). 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 (67% vs 0%; p=0.001) but not in CYC-treated group (12% vs 4%; p=0.38).

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.