Progress in treatment of ANCA-associated vasculitis

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-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

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.