Table 1

 Definitions for disease stages used for subclassification of patients with Wegener’s granulomatosis in clinical trials

Study groupClinical subgroupSystemic vasculitis outside ENT tract and lungThreatened vital organ functionOther definitionsSerum creatinine(µmol/l)Reference
ANCA, anti-neutrophil cytoplasmic antibody; CYC, cyclophosphamide; ENT, ear, nose and throat; EUVAS, European Vasculitis Study Group; VCRC, Vasculitis Clinical Research Consortium; WGET, Wegener’s Granulomatosis Etanercept Trial.
EUVASLocalisedNoNoNo constitutional symptoms, ANCA typically negative<120
Early systemicYesNoConstitutional symptoms present, ANCA-positive or -negative<120
GeneralisedYesYesANCA-positive<500Jayne et al2
SevereYesOrgan failureANCA-positive>500Jayne68
RefractoryYesYesRefractory to standard therapyAnyJayne68
WGET Research Group/VCRCLimitedAllowed, but not requiredNoNot severe⩽124, if haematuria, but no red blood cell casts presentWGET Research Group3
SevereYesYesOrgan- or life-threatening disease,implies need for remission induction with CYCAnyWGET Research Group3