Table 1

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

Clinical subgroup

Systemic vasculitis outside ENT and lung

Threatened vital organ function

Other definitions

Serum creatinine (╬╝mol/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


ANCA, antineutrophil cytoplasmic antibody; ENT, ear-nose-throat; EULAR, European League Against Rheumatism; WG, Wegener's granulomatosis. Adapted from [11] with permission.

Holle et al. Arthritis Research & Therapy 2011 13:224   doi:10.1186/ar3307