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 not requiring dialysis and/or other imminent vital organ failure† | Yes | + |
Severe renal | Renal requiring dialysis | Yes | + |
↵* Fever, night sweats, weight loss, malaise, and fatigue.
↵** Children with isolated proteinuria < 1 g/m2/day were not excluded from the early systemic group.
↵† Defined as alveolar hemorrhage, respiratory failure, mesenteric ischemia, scleritis, retinal hemorrhage, meningitis, stroke, cranial nerve palsy, sensory peripheral neuropathy, and mononeuritis multiplex. EUVAS: European Vasculitis Study; ANCA: antineutrophil cytoplasmic antibody.