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Table 1 Definition of disease stages in antineutrophil cytoplasmic antibody-associated vasculitis according to EULAR [8]

From: Conditions in subjects with rheumatic diseases: pulmonary manifestations of vasculitides

Clinical subgroup Systemic vasculitis outside ENT and lung Threatened vital organ function Other definitions Serum creatinine (μmol/l) Pulmonary manifestations
Localized No No No B-symptoms
ANCA typically negative
<120 Ulcerative bronchitis/bronchial stenosis in WG
Pulmonary masses 'granuloma' in WG
Early systemic Yes No B-symptoms
ANCA negative or positive
<120 As above
Generalized Yes Yes ANCA positive <500 As above
Pulmonary capillaritis, alveolitis
Severe Yes Organ failure ANCA positive >500 As above with organ failure
Refractory Yes Yes Refractory to standard therapy Any As above, resistant to standard therapy
  1. ANCA, antineutrophil cytoplasmic antibody; ENT, ear-nose-throat; EULAR, European League Against Rheumatism; WG, Wegener's granulomatosis. Adapted from [11] with permission.