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Table 2 European League Against Rheumatism (EULAR)-Definitions of AAV-Phenotypes (localized, early-systemic, generalized) and disease courses (serious, refractory) [42, 43]

From: Cerebral involvement in systemic vasculitides

Localized

upper and/or lower respiratory tract +/−

Early systemic

without impaired organ function or life-threatening disease +

Generalized disease

with impairment of renal or other organ function, Creatinine < 500 μmol/l +

Serious

renal or vital organ failure, Creatinine ≥500 μmol/l +

Refractory

progressive disease which does not respond to standard therapy +

  1. Abbreviations: + ANCA-positive,  ANCA-negative