Table 1.

Definitions for disease stages used for subclassification of parents with Wegner’s granulomatosis in clinical trials

Study	Clinical subgroup	Systemic vasculitis outside	Threatened vital organ function	Other definitions	Serum creatinine (μmol/L)	Reference
		ENT tract and lung
EUVAS	Localised	No	No	No constitutional symptoms, ANCA typically negative	<120
	Early systemic	Yes	No	Constitutional symptoms present, ANCA-positive or ANCA-negative	<120
	Generalised	Yes	Yes	ANCA-positive	<500	Jayne et al [4]
	Severe	Yes	Organ failure	ANCA-positive	>500	Jayne [5]
	Refractory	Yes	Yes	Refractory to standard therapy	Any	Jayne [5]
WGET Research Group/VCRC	Limited	Allowed, but not required	No	Not severe	≤124, if haematuria, but no red blood cell casts present	WGET Research Group [6]
	Severe	Yes	Yes	Organ- or life-threatening disease, implies need for remission induction with CYC	Any	WGET Research Group [6]

ANCA anti-neutrophil cytoplasmic antibody; CYC cyclophosphamide; ENT ear, nose and throat; EUVAS European Vasculitis Study Group; VCRC Vasculitis Clinical Research Consortium; WGET Wegner’s Granulomatosis Etanercept Trial