Prevalence and risk factors of relapse in patients with ANCA-associated vasculitis receiving cyclophosphamide induction: a systematic review and meta-analysis of large observational studies

Abstract

Background Clinical relapses are common in patients with ANCA-associated vasculitis (AAV). The aim of this systematic review was to estimate time-point prevalence and risk factors of relapse. Methods We searched PubMed, Embase, and Cochrane Library databases from their inception to March 30, 2020. Cohorts and post-hoc studies were included for the estimation of summary cumulative relapse rates (CRRs) and adjusted hazard ratios (aHRs) with 95% confidence intervals (CIs). Sensitivity and meta-regression analyses were also performed. Results Of the 42 eligible studies, 24 studies with 6236 participants were used for the pooled analyses of CRRs. The summary 1-year, 3-year, and 5-year CRRs were 0.12 (95% CI, 0.10–0.14), 0.33 (0.29–0.38), and 0.47 (0.42–0.52), respectively. In meta-regressions, the baseline age was positively associated with 1-year CRR. The proportion of granulomatosis with polyangiitis was positively associated with 5-year CRR. Twenty-eight studies with 5390 participants were used for the meta-analysis of risk factors for relapse, including a lower level of baseline serum creatine, proteinase 3 (PR3)-ANCA positivity at diagnosis, an ANCA rise, extrarenal organ involvement (including lung, cardiovascular, upper respiratory, and gastrointestinal involvement), intravenous (vs oral) cyclophosphamide induction, a shorter course of immunosuppressant maintenance, and maintenance with mycophenolate mofetil (vs azathioprine). Conclusions Our systematic review demonstrated that the 1-year, 3-year, and 5-year cumulative probabilities of relapse were ∼12%, 33%, and 47% in AAV patients receiving cyclophosphamide induction, respectively. Early identification of risk factors for relapse is helpful to the risk stratification of patients so as to achieve personalized treatment.

Clinical Practice Audit on the Management of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis in the Netherlands

“…as induction therapy. 19 Our study observed 19%, 22% and 24% hospitalization for serious infections during the first 6, 12 and 18 months and 7%, 27% and 38% disease relapses in 1, 3 and 5 years of follow-up respectively.…”

Clinical characteristics and long-term outcome of patients with gastrointestinal involvement in eosinophilic granulomatosis with polyangiitis

“…We compared the clinical data, treatment, and outcome of EGPA patients with GI involvement and EGPA patients without GI involvement. During follow-up, remission was defined as the absence of clinical systemic manifestations due to vasculitis ( 3 , 20 , 21 ), and relapse was defined as worsening disease manifestations requiring dose increase of glucocorticoids and/or addition or switch of immunosuppressive medication ( 21 , 22 ).…”

Diagnose und Therapie der Granulomatose mit Polyangiitis und mikroskopische Polyangiitis – 2023: Konsens-Empfehlungen der Österreichischen Gesellschaften für Nephrologie (ÖGN) & Rheumatologie (ÖGR)

“…Bei der Mehrzahl der Patient*innen mit AAV kommt es nach einer wirksamen Induktionstherapie zu einer Remission. Jedoch kommt es bei einer erheblichen Anzahl (fast 50 %) [ 51 ] der Patient*innen innerhalb von fünf Jahren nach der Diagnose zu einem Rezidiv, und dies geht mit einer Zunahme der krankheits- und therapiebezogenen Morbidität und Mortalität einher [ 52 ]. Eine Subanalyse der RAVE-Studie zeigte, dass RTX bei Patient*innen mit Rezidiv, insbesondere bei PR3-ANCA-Vaskulitis, gegenüber CYC/AZA überlegen ist [ 40 ].…”