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
| |