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. 2022 Dec 15:6:100178.
doi: 10.1016/j.jtauto.2022.100178. eCollection 2023.

Adding low dose cyclophosphamide to rituximab for remission-induction may prolong relapse-free survival in patients with ANCA vasculitis: A retrospective study

Renée Ysermans  1 , Matthias H Busch  1 , Joop P Aendekerk  1 , Jan G M C Damoiseaux  2 , Pieter van Paassen  1
Affiliations

Adding low dose cyclophosphamide to rituximab for remission-induction may prolong relapse-free survival in patients with ANCA vasculitis: A retrospective study

Renée Ysermans et al. J Transl Autoimmun. .

Abstract

Objective: Rituximab (RTX) and cyclophosphamide (CYC) are effective remission-induction therapies in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). However, combining these therapies may favor prognosis in patients with a major disease presentation. We conducted a retrospective study to compare patients treated with a combination of RTX and low dose CYC (RTX-CYC) or with RTX only, both followed by tailored maintenance with RTX, with regard to long-term outcomes.

Methods: Patients treated in the Maastricht University Medical Center between March 2007 and January 2019, were screened for eligibility. The primary outcome variable was major relapse rate after two and five years. Secondary outcome variables were clinical data and laboratory parameters.

Results: Of the 246 screened patients, 34 received RTX-CYC and 28 RTX only for remission-induction. All patients were followed for at least two years, with a median follow-up of 48 months (IQR 24-60). At baseline, renal involvement was more prevalent in the RTX-CYC patients (85% vs. 61%, P = 0.028). Major relapse rates within two years, but not after five years, were significantly lower in the RTX-CYC group (3% vs. 24%, P = 0.032). The rate of infections, hypogammaglobulinemia, end-stage renal disease, malignancies, and mortality did not differ after two and five years.

Conclusion: Adding low dose CYC to RTX is safe and may prevent major relapses in patients with severe AAV in the first two years after remission-induction. Randomized controlled trials that compare the efficacy and safety between RTX and a combination of RTX with CYC are needed.

Keywords: ANCA; Cyclophosphamide; Outcome; Relapse; Rituximab; Vasculitis.

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Conflict of interest statement

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Figures

Fig. 1
Fig. 1
Flowchart of included patients. Abbreviations: AAV = anti-neutrophil cytoplasmic antibody-associated vasculitis, RTX = rituximab, CYC = cyclophosphamide.
Fig. 2
Fig. 2
Cumulative percentage of patients in remission (A) and with relapse-free survival (B). Abbreviations: RTX = rituximab, CYC = cyclophosphamide. + = event/censored.
Fig. 3
Fig. 3
Cumulative percentage of patients with infections (A) and of patient survival (B). Abbreviations: RTX = rituximab, CYC = cyclophosphamide. + = event/censored.
See this image and copyright information in PMC

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