Table 2

 Use of Birmingham Vasculitis Activity Score and Birmingham Vasculitis Activity Score for Wegener’s granulomatosis in clinical trials

TrialDetailsInterventionOutcomeBVAS at entryDisease assessment scores used
AASV, antineutrophil cytoplasmic antibody-associated systemic vasculitis; Aza, azathioprine; BVAS, Birmingham Vasculitis Activity Score; BVAS/WG, Birmingham Vasculitis Activity Score for Wegener’s granulomatosis; CR, complete remission; Cryo, cryoglobulinaemia; CS, corticosteroids; CSS, Churg–Strauss syndrome; CYC, cyclophosphamide; DEI, Disease Extent Index; DSG, deoxyspergualin; FFS, Five Factor score; iv Ig, intravenous immunoglobulin; LN, lupus nephritis; MMF, mycophenolate mofetil; MPA, microscopical polyangiitis; MTX, methotrexate; PP, plasmapheresis; PR, partial remission; RCT, randomised controlled trial; RV, rheumatoid arthritis vasculitis; SF-36, Short Form 36; SLEDAI, Systemic Lupus Erythematosus Disease Activity Index; SNVDI, Systemic Necrotising Vasculitis Damage Index; VDI, Vasculitis Damage Index; WG, Wegener’s granulomatosis.
Keogh et al,322006Prospective, open-label,10 WGRituximab for refractory vasculitisCR at 3 months 100%Median 6BVAS/WG, SF-36
WGET,33 2005Double-blind RCT, 180 WGStandard treatment+etanercept v placebo for remission maintenanceNo major difference in sustained remission rates (69.7% v 75.3%)Mean 6.5 and 7.5BVAS/WG, VDI, SF-36
De Groot et al,22 2005RCT, 100 new onset AASVMTX v CYC for remission inductionRemission 6 months, MTX 89.8%, CYC 93.5%Median 15BVAS 1+2, DEI, VDI
Benenson et al,23 2005Open-label, 4 WG,2 LNHigh dose intravenous Aza in refractory WG or LN2 CR, 2 PR (LN),2 no responseRange 2–14BVAS, SLEDAI
Joy et al,31 2005Prospective, open-label,12 AASVMMF for relapsing or persistent diseaseReduction in BVAS at 24 and 52 weeksMean 9.1BVAS 1+2
Booth et al,272004Prospective, open-label,32 AASVAddition of infliximab for remission induction in new and persistent diseaseRemission 88%Mean 12.3BVAS 1+2
Metzler et al,28 2004Prospective, open-label,20 WGLeflunomide for remission maintenance1 major and 8 minor relapses; median follow-up 1.75 yearsMedian 0DEI, BVAS, SF-36
Danieli et al,242004Non-randomised, prospective, historic controls, 18 CSSAddition of iv Ig+PP v standard treatment alone in new CSSRemission 12 months, iv Ig 100%, control 44%Range 13–31BVAS, FFS, Rankin score, SNVDI
Jayne et al,252003RCT, 155 new-onset AASVAza v CYC for remission maintenanceRelapse 18 months, Aza 15.5%, CYC 13.7%Mean 18BVAS 1+2, DEI, VDI, SF-36
Birck et al,302003Prospective, open–label, 19 WG,1 MPADSG in refractory disease6 CR and 8 PR after 6 monthsRange 3–25BVAS, VDI, SF-36
Bartolucci et al,29 2002Prospective, open-label,7 WG, 2 RV, 1 CryoInfliximab+CS for refractory vasculitis5 CR and 5 PR after 6 monthsMean 9.1BVAS
Stone et al,202001Prospective, open-label,20 WGStandard treatment+etanercept for relapsing and persistent disease80% achieved BVAS/WG at some pointMean BVAS/WG 3.6BVAS/WG
Jayne et al,262000Double-blind, placebo-controlled, 34 AASVStandard treatment+iv Ig v placebo for persistent diseaseTherapeutic response at 3 months, iv Ig 82.4%; placebo 35.3%Mean 6.1 and 5.4BVAS