Table 1

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

Study group	Clinical subgroup	Systemic vasculitis outside ENT tract and lung	Threatened vital organ function	Other definitions	Serum 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.
EUVAS	Localised	No	No	No constitutional symptoms, ANCA typically negative	<120
	Early systemic	Yes	No	Constitutional symptoms present, ANCA-positive or -negative	<120
	Generalised	Yes	Yes	ANCA-positive	<500	Jayne et al²
	Severe	Yes	Organ failure	ANCA-positive	>500	Jayne⁶⁸
	Refractory	Yes	Yes	Refractory to standard therapy	Any	Jayne⁶⁸
WGET Research Group/VCRC	Limited	Allowed, but not required	No	Not severe	⩽124, if haematuria, but no red blood cell casts present	WGET Research Group³
	Severe	Yes	Yes	Organ- or life-threatening disease,implies need for remission induction with CYC	Any	WGET Research Group³