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The REACT score predicts relapse in ANCA-associated vasculitis

Presented by
Dr Gianmarco Lugli, Meyer Children's Hospital IRCCS, Florence, Italy
Conference
ERA 2024
Doi
https://doi.org/10.55788/1bcf7f9d
A new risk score called REACT can predict relapses following cyclophosphamide in participants with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, data from a multinational retrospective cohort study suggests.

“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.

  1. 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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