Background
Clinical tool | Description |
---|---|
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 | |
BVAS for Wegener’s Granulomatosis (WG) | |
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) | |
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]. |
Methods
Results
Respondent characteristics
Classification criteria
Criteria | Familiar (%) | Using (%) |
---|---|---|
EULAR/PRINTO/PRES 2008 criteria [9] | 78 | 57 |
ANCA (PR3/MPO) | NDb
| 57 |
72 | 29 | |
CHCC 1994 [10] | 62 | 32 |
CHCC 2012 [1] | 47 | |
EMA classification algorithm (2007) [22] | 18 | 5 |
Pediatric Modification of EMA classification algorithm (2012) [21] | ||
Informal criteria | NDb
| 19 |
Other formal criteria | NDb
| 1 |
Disease severity, activity, and damage
Parameter | Scoring tool | Respondents (%) |
---|---|---|
Severityb
| PVAS [12] | 41 |
BVAS [11] | 20 | |
BVAS for WG [13] | 19 | |
EUVAS Severity Score [41] | 11 | |
Five Factor Score [42] | 8 | |
WGET Severity Score [43] | 7 | |
Disease Extent Index [44] | 1 | |
Other | 1 | |
Never formally assess | 41 | |
Activityc
| PVAS [12] | 38 |
Physician’s Global Assessment | 29 | |
BVAS [11] | 19 | |
BVAS for WG [13] | 13 | |
BVAS version 3 [39] | 6 | |
Other tool | 0 | |
Never formally assess | 37 | |
Damagec
| Pediatric VDI [15] | 31 |
VDI [14] | 10 | |
AAV Index of Damage [45] | 3 | |
Combined Damage Assessment Index [46] | 1 | |
Other | 0 | |
Never formally assess | 64 |
Parameter | Rationale against use of tool | Respondents (%) |
---|---|---|
Severityb
| Use of histopathological findings | 53 |
No effect on management | 34 | |
Lack of familiarity with tools | 26 | |
No value added beyond clinical judgment | 24 | |
Inconvenience | 16 | |
Other | 5.3 | |
Activityc
| Lack of familiarity with tools | 47 |
Inconvenience | 43 | |
No effect on management | 22 | |
Lack of applicability of tools for adults | 20 | |
No value added beyond clinical judgment | 18 | |
Damaged
| Lack of familiarity with tools | 58 |
Inconvenience | 32 | |
Lack of applicability of tools for adults | 20 | |
No effect on management | 18 | |
No value added beyond clinical judgment | 12 |
Treatment guidelines
Treatment practices
Indication | Agent | Respondents (%) |
---|---|---|
Induction agent in severe diseaseb
| Cyclophosphamide | 66 |
Rituximab | 31 | |
Other | 2 | |
Induction agent if not using CYC or RTX (e.g. for less severe disease)c
| Methotrexate | 40 |
Azathioprine | 30 | |
Mycophenolate | 25 | |
Other | 4 | |
Maintenance therapyd
| Azathioprine | 45 |
Methotrexate | 23 | |
Mycophenolate | 18 | |
Rituximab | 11 | |
Other | 3 |