Table 1A.

The EUVAS clinical subgroupings of ANCA-associated vasculitis based on extent and severity of disease.

Subgroup	Organ Involvement	Constitutional Symptoms^*	ANCA Status
Localized	Upper and/or lower respiratory tract	No	±
Early systemic	Any except renal or imminent vital organ failure^†	Yes	Usually +
Generalized	Renal with serum creatinine level < 500 μmol/l and/or other imminent vital organ failure^†	Yes	+
Severe renal	Renal with serum creatinine level > 500 μmol/l	Yes	+
Refractory	Progressive disease despite therapy with corticosteroids and cyclophosphamide	Yes	±

↵* Fever, night sweats, weight loss, malaise, and fatigue.
↵† Includes progressive lung, eye, nervous system, or gastrointestinal vasculitis. EUVAS: European Vasculitis Study; ANCA: antineutrophil cytoplasmic antibody.