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 |