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

Localised

One site affected, often upper respiratory tract

(Co-trimoxazole^a)

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