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Table 2 EUVAS disease categorisation of and randomised controlled trials in AAV according to disease severity

From: Progress in treatment of ANCA-associated vasculitis

Category Definition Induction Trial Maintenance Trial
Localised One site affected, often upper respiratory tract (Co-trimoxazolea)   Co-trimoxazole Stegeman and colleagues [57]
Early systemic Any disease, without imminent vital organ failure MTX or CYC + GC NORAM [32] Co-trimoxazole Stegeman and colleagues [57]
Generalised Renal or other organ threatening disease, creatinine <500 μmol/l CYC+ GC CYCLOPS [30] AZA + GC CYCAZAREM [28]
   RTX + GC RAVE [43] MTX + GC WEGENT [55]
   MMF + GC Hu and colleagues [46] Leflunomide Metzler and colleagues [56]
     AZA or MMF + GC IMPROVE [59]
Severe Renal or other vital organ failure, creatinine >500 μmol/l CYC or RTX + GC RITUXVAS [42] AZA or MMF + GC IMPROVE [59]
   PEX MEPEX [34]   
Refractory Progressive disease unresponsive to steroids and cyclophosphamide IVIg nonrandomised - RTX, DSG, MMF, ATG, IFX, HSCT, ALM Jayne and colleagues [65] No consensus  
  1. AAV, autoantibodies to neutrophil cytoplasmic antigen-associated vasculitis; ALM, alemtuzumab; ATG, anti-thymocyte globulin; AZA, azathioprine; CYC, cyclophosphamide; DSG, gusperimus; EUVAS, European Vasculitis Study Group; GC, glucocorticoids; HSCT, haemopoetic stem cell transplantation; IFX, infliximab; IVIg, intravenous immunoglobulin; MMF, mycophenolate mofetil; MTX, methotrexate; PEX, plasma exchange; RTX, rituximab. aTrial proposed but not conducted.