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Table 1 Disease Severity Definitions

From: Hallmark trials in ANCA-associated vasculitis (AAV) for the pediatric rheumatologist

Study Group

Clinical Subgroup

Systemic Vasculitis Outside Ears, Nose, Throat and Lungs

Threatened Vital Organ Function

Other Definitions

Serum Creatinine (umol/L)

EUVAS

Localized

No

No

No constitutional symptoms, ANCA typically negative

< 120

Early Systemic

Yes

No

Constitutional symptoms present, ANCA-positive or negative

< 120

Generalized

Yes

Yes

ANCA-positive

< 500

Severe

Yes

Organ Failure

ANCA-positive

> 500

Refractory

Yes

Yes

Refractory to standard therapy

Any

WGET Research Group

Limited

Allowed, but not required

No

Not severe

< 124, if hematuria, but no red blood cell casts present

Severe

Yes

Yes

Organ- or life-threatening disease

Any

  1. From Hellmich et al., EULAR Recommendations for conducting clinical studies and/or clinical trials in systemic vasculitis: focus on anti-neutrophil cytoplasm antibody-associated vasculitis. [12]