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