AIM: Currently, several different instruments are used to measure disease activity and extent in clinical trials of anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis, leading to division among investigative groups and difficulty comparing study results. An exercise comparing six different vasculitis instruments was performed. METHODS: A total of 10 experienced vasculitis investigators from 5 countries scored 20 cases in the literature of Wegener granulomatosis or microscopic polyangiitis using 6 disease assessment tools: the Birmingham Vasculitis Activity Score (BVAS), The BVAS for Wegener granulomatosis (BVAS/WG), BVAS 2003, a Physician Global Assessment (PGA), the Disease Extent Index (DEI) and the Five Factor Score (FFS). Five cases were rescored by all raters. RESULTS: Reliability of the measures was extremely high (intraclass correlations for the six measures all = 0.98). Within each instrument, there were no significant differences or outliers among the scores from the 10 investigators. Test/retest reliability was high for each measure: range = 0.77 to 0.95. The scores of the five acute activity measures correlated extremely well with one another. CONCLUSIONS: Currently available tools for measuring disease extent and activity in ANCA-associated vasculitis are highly correlated and reliable. These results provide investigators with confidence to compare different clinical trial data and helps form common ground as international research groups develop new, improved and universally accepted vasculitis disease assessment instruments.
AIM: Currently, several different instruments are used to measure disease activity and extent in clinical trials of anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis, leading to division among investigative groups and difficulty comparing study results. An exercise comparing six different vasculitis instruments was performed. METHODS: A total of 10 experienced vasculitis investigators from 5 countries scored 20 cases in the literature of Wegener granulomatosis or microscopic polyangiitis using 6 disease assessment tools: the Birmingham Vasculitis Activity Score (BVAS), The BVAS for Wegener granulomatosis (BVAS/WG), BVAS 2003, a Physician Global Assessment (PGA), the Disease Extent Index (DEI) and the Five Factor Score (FFS). Five cases were rescored by all raters. RESULTS: Reliability of the measures was extremely high (intraclass correlations for the six measures all = 0.98). Within each instrument, there were no significant differences or outliers among the scores from the 10 investigators. Test/retest reliability was high for each measure: range = 0.77 to 0.95. The scores of the five acute activity measures correlated extremely well with one another. CONCLUSIONS: Currently available tools for measuring disease extent and activity in ANCA-associated vasculitis are highly correlated and reliable. These results provide investigators with confidence to compare different clinical trial data and helps form common ground as international research groups develop new, improved and universally accepted vasculitis disease assessment instruments.
Authors: J H Stone; G S Hoffman; P A Merkel; Y I Min; M L Uhlfelder; D B Hellmann; U Specks; N B Allen; J C Davis; R F Spiera; L H Calabrese; F M Wigley; N Maiden; R M Valente; J L Niles; K H Fye; J W McCune; E W St Clair; R A Luqmani Journal: Arthritis Rheum Date: 2001-04
Authors: Peter A Merkel; Philip Seo; Peter Aries; Tuhina Neogi; Alexandra Villa-Forte; Maarten Boers; David Cuthbertson; David T Felson; Bernhard Hellmich; Gary S Hoffman; David R Jayne; Cees G M Kallenberg; Jeffrey Krischer; Alfred Mahr; Eric L Matteson; Ulrich Specks; Raashid Luqmani; John Stone Journal: J Rheumatol Date: 2005-12 Impact factor: 4.666
Authors: L Guillevin; J F Cordier; F Lhote; P Cohen; B Jarrousse; I Royer; P Lesavre; C Jacquot; P Bindi; P Bielefeld; J F Desson; F Détrée; A Dubois; E Hachulla; B Hoen; D Jacomy; C Seigneuric; D Lauque; M Stern; M Longy-Boursier Journal: Arthritis Rheum Date: 1997-12
Authors: Philip Seo; Yuan-I Min; Janet T Holbrook; Gary S Hoffman; Peter A Merkel; Robert Spiera; John C Davis; Steven R Ytterberg; E William St Clair; W Joseph McCune; Ulrich Specks; Nancy B Allen; Raashid A Luqmani; John H Stone Journal: Arthritis Rheum Date: 2005-07
Authors: David Jayne; Niels Rasmussen; Konrad Andrassy; Paul Bacon; Jan Willem Cohen Tervaert; Jolanta Dadoniené; Agneta Ekstrand; Gill Gaskin; Gina Gregorini; Kirsten de Groot; Wolfgang Gross; E Christiaan Hagen; Eduardo Mirapeix; Erna Pettersson; Carl Siegert; Alberto Sinico; Vladimir Tesar; Kerstin Westman; Charles Pusey Journal: N Engl J Med Date: 2003-07-03 Impact factor: 91.245
Authors: L Guillevin; F Lhote; M Gayraud; P Cohen; B Jarrousse; O Lortholary; N Thibult; P Casassus Journal: Medicine (Baltimore) Date: 1996-01 Impact factor: 1.889
Authors: Peter C Grayson; Paul A Monach; Christian Pagnoux; David Cuthbertson; Simon Carette; Gary S Hoffman; Nader A Khalidi; Curry L Koening; Carol A Langford; Kathleen Maksimowicz-McKinnon; Philip Seo; Ulrich Specks; Steven R Ytterberg; Peter A Merkel Journal: Rheumatology (Oxford) Date: 2014-11-17 Impact factor: 7.580
Authors: Marta Casal Moura; Maria V Irazabal; Alfonso Eirin; Ladan Zand; Sanjeev Sethi; Bijan J Borah; Jeffrey L Winters; James P Moriarty; Rodrigo Cartin-Ceba; Alvise Berti; Misbah Baqir; Gwen E Thompson; Ashima Makol; Kenneth J Warrington; Viengneesee Thao; Ulrich Specks; Fernando C Fervenza Journal: J Am Soc Nephrol Date: 2020-08-21 Impact factor: 10.121
Authors: Jan Henrik Schirmer; Peer M Aries; Kirsten de Groot; Bernhard Hellmich; Julia U Holle; Christian Kneitz; Ina Kötter; Peter Lamprecht; Ulf Müller-Ladner; Eva Reinhold-Keller; Christof Specker; Michael Zänker; Frank Moosig Journal: Z Rheumatol Date: 2017-11 Impact factor: 1.372
Authors: Francisco Silva; Ulrich Specks; Sanjay Kalra; Marie C Hogan; Nelson Leung; Sanjeev Sethi; Fernando C Fervenza Journal: Clin J Am Soc Nephrol Date: 2010-01-21 Impact factor: 8.237
Authors: Peter A Merkel; Karen Herlyn; Alfred D Mahr; Tuhina Neogi; Philip Seo; Michael Walsh; Maarten Boers; Raashid Luqmani Journal: J Rheumatol Date: 2009-10 Impact factor: 4.666
Authors: Tanaz A Kermani; David Cuthbertson; Simon Carette; Gary S Hoffman; Nader A Khalidi; Curry L Koening; Carol A Langford; Kathleen McKinnon-Maksimowicz; Carol A McAlear; Paul A Monach; Philip Seo; Kenneth J Warrington; Steven R Ytterberg; Peter A Merkel; Eric L Matteson Journal: J Rheumatol Date: 2016-04-01 Impact factor: 4.666