Category | Definition | Induction | Trial | Maintenance | Trial |
---|---|---|---|---|---|
Localised | One site affected, often upper respiratory tract | (Co-trimoxazolea) | Â | 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 | Â |