https://doi.org/10.55788/1bcf7f9d
“Cyclophosphamide use is associated with sustained remission induction in ANCA-associated vasculitis. Several clinical factors, however, likely co-affect this risk for relapse”, started Dr Gianmarco Lugli (Meyer Children's Hospital IRCCS, Florence, Italy) and introduced the reasoning for performing a retrospective study, using three national cohorts from Italy, Ireland, and Spain, to establish a relapse risk score for ANCA-associated vasculitis patients.
The retrospective study included 593 participants (261 with microscopic polyangiitis, 259 with granulomatosis with polyangiitis and 73 with eosinophilic granulomatosis with polyangiitis), of whom 552/593 (93%) had remission, and 271/552 (49%) developed relapse. Cohort participants were eligible for inclusion if they were adults with a diagnosis of granulomatosis with polyangiitis, microscopic polyangiitis, or eosinophilic granulomatosis with polyangiitis (with a Five-Factor Score >0), received induction with cyclophosphamide (both oral and intravenous were eligible) and had a follow-up of at least 12 months. Relapse was defined as at least 1 new vasculitis event after remission of ≥3 months. [1].
Although organ involvement varied among participants with different ANCA-associated vasculitis, kidney involvement was present in 80% of the cohort. The median cohort relapse-free survival time was over 24 months; however, participants with eosinophilic granulomatosis with polyangiitis had longer relapse-free survival than those with microscopic polyangiitis and granulomatosis with polyangiitis (P<0.0001). Multivariate analyses showed that IgG class ANCA directed to proteinase 3 (PR3) levels, intravenous cyclophosphamide, cardiovascular involvement, arthralgias/arthritis and the absence of rapidly progressive glomerulonephritis were all independent predictors of relapse. Based on these data, a “Relapse Evaluation And Cyclophosphamide Treatment (REACT) score” (range 0–7) was created, whereby all independent predictors were weighted with 1 point, except intravenous cyclophosphamide and cardiovascular involvement, which received 2 points. REACT scores identified 138 (27%) of participants as low-risk (scores 0–1), 252 (59%) as moderate-risk (scores 2–3), and 115 (22%) as high-risk (scores ≥4) categories which could significantly predict relapse-free survival on Kaplan-Meier analysis.
“The REACT score could be employed at diagnosis to predict the risk of relapse, but the score will require future external validation”, concluded Dr Lugli.
- Lugli G, et al. Development of a relapse risk score in patients with ANCA-associated vasculitis treated with cyclophosphamide induction. Abstract #1484, ERA 2024, 23–26 May, Stockholm, Sweden.
Medical writing support was provided by Mihai Surducan, PhD.
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