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 |