Table 1 Definitions of clinical tools included in survey questions

Birmingham Vasculitis Activity Score (BVAS)

Scoring tool designed to document new or worseningfeatures of clinically active AAV. Items are categorized into 9 groups by organ system [11].

BVAS Version 3

2009 update to BVAS [11, 39].

BVAS for Wegener’s Granulomatosis (WG)

Modification of BVAS that removes features unlikely to occur in GPA [13, 40].

Pediatric Vasculitis Activity Score (PVAS)

Pediatric version of BVAS preliminarily validated in children [12].

EUVAS Severity Score

Classification system for AAV based on disease extent and severity. Subgroups include localized, early systemic, generalized, severe renal, and refractory disease [41].

Five Factor Score

Scoring tool based on factors associated with poor prognosis: cardiac symptoms, gastrointestinal involvement, renal insufficiency, proteinuria, and central nervous system involvement [42].

Wegener’s Granulomatosis Etanercept Trial (WGET) Severity Score

Sub-classification based on modified ACR criteria for limited versus extensive disease [43].

Disease Extent Index (DEI)

Tool for scoring disease activity based on organ system, with distinct domains from those included in BVAS [44].

Physician’s Global Assessment (PGA)

Physician’s global assessment of disease activity on a 10 cm visual analogue scale.

Vasculitis Damage Index (VDI)

Scoring tool used to record damage due to disease or treatment. Items are categorized into 11 groups by organ system with binary scoring [14].

Pediatric VDI (PVDI)

Pediatric modification of the adult VDI; not yet formally validated in children [14, 15].

AAV Index of Damage (AVID)

Tool for grading disease- or treatment-associated damage, with weighting of items based on potential morbidity and mortality [45].

Combined Damage Assessment Index

Combination of items from AVID and VDI, divided into 17 organ-based categories [46].