Modification and validation of the Birmingham Vasculitis Activity Score (version 3)

Background: Comprehensive multisystem clinical assessment using the Birmingham Vasculitis Activity score (BVAS) is widely used in therapeutic studies of systemic vasculitis. Extensive use suggested a need to revise the instrument. The previous version of BVAS has been revised, according to usage and reviewed by an expert committee. Objective: To modify and validate version 3 of the BVAS in patients with systemic vasculitis. Methods: The new version of BVAS was tested in a prospective cross-sectional study of patients with vasculitis. Results: The number of items was reduced from 66 to 56. The subscores for new/worse disease and persistent disease were unified. In 313 patients with systemic vasculitis, BVAS(v.3) correlated with treatment decision (Spearman’s rs = 0.66, 95% CI 0.59 to 0.72), BVAS1 of version 2 (rs = 0.94, 95% CI 0.92 to 0.96), BVAS2 of version 2 in patients with persistent disease (rs = 0.60, 95% CI 0.21 to 0.83), C-reactive protein levels (rs = 0.43, 95% CI 0.31 to 0.54), physician’s global assessment (rs = 0.91, 95% CI 0.89 to 0.93) and vasculitis activity index (rs = 0.88, 95% CI 0.86 to 0.91). The intraclass correlation coefficients for reproducibility and repeatability were 0.96 (95% CI 0.95 to 0.97) and 0.96 (95% CI 0.92 to 0.97), respectively. In 39 patients assessed at diagnosis and again at 3 months, the BVAS(v.3) fell by 17 (95% CI 15 to 19) units (p<0.001, paired t test). Conclusion: BVAS(v.3) demonstrates convergence with BVAS(v.2), treatment decision, physician global assessment of disease activity, vasculitis activity index and C-reactive protein. It is repeatable, reproducible and sensitive to change. The new version of BVAS is validated for assessment of systemic vasculitis.

[1]  R. Luqmani,et al.  Outpatient assessment of systemic vasculitis. , 2007, Best practice & research. Clinical rheumatology.

[2]  D. Abramowicz,et al.  Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. , 2007, Journal of the American Society of Nephrology : JASN.

[3]  H. Raspe,et al.  EULAR recommendations for conducting clinical studies and/or clinical trials in systemic vasculitis: focus on anti-neutrophil cytoplasm antibody-associated vasculitis , 2006, Annals of the rheumatic diseases.

[4]  P. Bacon,et al.  Development of comprehensive disease assessment in systemic vasculitis. , 2007, Annals of the rheumatic diseases.

[5]  R. Luqmani,et al.  Remission in antineutrophil cytoplasmic antibody-associated systemic vasculitis. , 2006, Clinical and experimental rheumatology.

[6]  U. Maggiore,et al.  Outcome and prognostic factors during the course of primary small-vessel vasculitides. , 2006, The Journal of rheumatology.

[7]  O. Bakoush,et al.  Urine IgM excretion predicts outcome in ANCA-associated renal vasculitis. , 2006, Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association.

[8]  F. J. van der Woude,et al.  Serial ANCA determinations for monitoring disease activity in patients with ANCA-associated vasculitis: systematic review. , 2006, American journal of kidney diseases : the official journal of the National Kidney Foundation.

[9]  Jianwen Cai,et al.  Predictors of Relapse and Treatment Resistance in Antineutrophil Cytoplasmic AntibodyAssociated Small-Vessel Vasculitis , 2005, Annals of Internal Medicine.

[10]  L. Mouthon,et al.  Deaths Occurring During the First Year After Treatment Onset for Polyarteritis Nodosa, Microscopic Polyangiitis, and Churg-Strauss Syndrome: A Retrospective Analysis of Causes and Factors Predictive of Mortality Based on 595 Patients , 2005, Medicine.

[11]  C. Feighery,et al.  Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. , 2005, Arthritis and rheumatism.

[12]  P. Merkel,et al.  Damage caused by Wegener's granulomatosis and its treatment: prospective data from the Wegener's Granulomatosis Etanercept Trial (WGET). , 2005, Arthritis and rheumatism.

[13]  D. Scott,et al.  Primary systemic vasculitis: clinical features and mortality. , 2005, QJM : monthly journal of the Association of Physicians.

[14]  P. Bacon.  The spectrum of Wegener's granulomatosis and disease relapse. , 2005, The New England journal of medicine.

[15]  F Guillemin,et al.  EULAR standardised operating procedures for the elaboration, evaluation, dissemination, and implementation of recommendations endorsed by the EULAR standing committees , 2004, Annals of the rheumatic diseases.

[16]  T. Colby,et al.  Antineutrophil cytoplasmic autoantibodies against the murine homolog of proteinase 3 (Wegener autoantigen) are pathogenic in vivo. , 2004, Blood.

[17]  C. Stegeman,et al.  Positive classic antineutrophil cytoplasmic antibody (C-ANCA) titer at switch to azathioprine therapy associated with relapse in proteinase 3-related vasculitis. , 2004, Arthritis and rheumatism.

[18]  L. Mouthon,et al.  Predicting mortality in systemic Wegener's granulomatosis: a survival analysis based on 93 patients. , 2004, Arthritis and rheumatism.

[19]  K. Farrington,et al.  Outcome in glomerulonephritis due to systemic small vessel vasculitis: effect of functional status and non-vasculitic co-morbidity. , 2004, Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association.

[20]  K. De Groot,et al.  Churg Strauss syndrome--successful induction of remission with methotrexate and unexpected high cardiac and pulmonary relapse ratio during maintenance treatment. , 2004, Clinical and experimental rheumatology.

[21]  D. Scott,et al.  Investigation of quality of life, mood, pain, disability, and disease status in primary systemic vasculitis. , 2003, Arthritis and rheumatism.

[22]  A. Bladström,et al.  High proteinase 3-anti-neutrophil cytoplasmic antibody (ANCA) level measured by the capture enzyme-linked immunosorbent assay method is associated with decreased patient survival in ANCA-associated vasculitis with renal involvement. , 2003, Journal of the American Society of Nephrology : JASN.

[23]  V. Tesar,et al.  A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. , 2003, The New England journal of medicine.

[24]  C. Langford,et al.  Use of a cyclophosphamide-induction methotrexate-maintenance regimen for the treatment of Wegener's granulomatosis: extended follow-up and rate of relapse. , 2003, The American journal of medicine.

[25]  L. Mouthon,et al.  Treatment of polyarteritis nodosa and microscopic polyangiitis with poor prognosis factors: a prospective trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in sixty-five patients. , 2003, Arthritis and rheumatism.

[26]  J. M. Drexler,et al.  15-Deoxyspergualin in patients with refractory ANCA-associated systemic vasculitis: a six-month open-label trial to evaluate safety and efficacy. , 2003, Journal of the American Society of Nephrology : JASN.

[27]  W. Koldingsnes,et al.  Baseline features and initial treatment as predictors of remission and relapse in Wegener's granulomatosis. , 2003, The Journal of rheumatology.

[28]  P. Heeringa,et al.  Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice. , 2002, The Journal of clinical investigation.

[29]  G. Sobue,et al.  Mortality and morbidity in peripheral neuropathy associated Churg-Strauss syndrome and microscopic polyangiitis. , 2002, The Journal of rheumatology.

[30]  W. Koldingsnes,et al.  Predictors of survival and organ damage in Wegener's granulomatosis. , 2002, Rheumatology.

[31]  C. Kallenberg,et al.  Patients' perceptions of the effects of systemic lupus erythematosus on health, function, income, and interpersonal relationships: a comparison with Wegener's granulomatosis. , 2002, Arthritis and rheumatism.

[32]  L. Bostad,et al.  Clinical Outcome of Patients with Wegener’s Granulomatosis Treated with Plasma Exchange , 2002, Blood Purification.

[33]  R. Solans,et al.  Churg-Strauss syndrome: outcome and long-term follow-up of 32 patients. , 2001, Rheumatology.

[34]  L. Bostad,et al.  Renal histopathology and clinical course in 94 patients with Wegener's granulomatosis. , 2001, Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association.

[35]  P. Merkel,et al.  A disease-specific activity index for Wegener's granulomatosis: modification of the Birmingham Vasculitis Activity Score. International Network for the Study of the Systemic Vasculitides (INSSYS). , 2001, Arthritis and rheumatism.

[36]  L. Guillevin,et al.  Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: analysis of four prospective trials including 278 patients. , 2001, Arthritis and rheumatism.

[37]  E. Hachulla,et al.  Granulomatose de Wegener du sujet âgé , 2001 .

[38]  C. Le Jeunne,et al.  [Churg-Strauss syndrome. Retrospective study of 20 cases]. , 2001, Bulletin de l'Academie nationale de medecine.

[39]  W. Samtleben,et al.  [Wegener granulomatosis and microscopic polyangiitis. Diagnostic and clinical results in 54 patients with long-term follow-up]. , 2000, Deutsche medizinische Wochenschrift.

[40]  P. Limburg,et al.  Prediction of relapses in Wegener's granulomatosis by measurement of antineutrophil cytoplasmic antibody levels: a prospective study. , 2000, Arthritis and rheumatism.

[41]  D. Adu,et al.  Intravenous immunoglobulin for ANCA-associated systemic vasculitis with persistent disease activity. , 2000, QJM : monthly journal of the Association of Physicians.

[42]  L. Guillevin,et al.  Microscopic Polyangiitis with Alveolar Hemorrhage A Study of 29 Cases and Review of the Literature , 2000, Medicine.

[43]  M. Heller,et al.  An interdisciplinary approach to the care of patients with Wegener's granulomatosis: long-term outcome in 155 patients. , 2000, Arthritis and rheumatism.

[44]  L. Vatten,et al.  Wegener's granulomatosis: clinical course in 108 patients with renal involvement. , 2000, Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association.

[45]  J. Amouroux,et al.  Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. , 1999, Arthritis and rheumatism.

[46]  L. Guillevin,et al.  Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. , 1999, Medicine.

[47]  M. Haubitz,et al.  Intravenous pulse administration of cyclophosphamide versus daily oral treatment in patients with antineutrophil cytoplasmic antibody-associated vasculitis and renal involvement: a prospective, randomized study. , 1998, Arthritis and rheumatism.

[48]  M. Haubitz,et al.  Survival and vasculitis activity in patients with end-stage renal disease due to Wegener's granulomatosis. , 1998, Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association.

[49]  C. Pusey,et al.  Outcome of renal replacement therapy in antineutrophil cytoplasmic antibody-associated systemic vasculitis. , 1998, Journal of the American Society of Nephrology : JASN.

[50]  K. De Groot,et al.  Induction of remission in Wegener's granulomatosis with low dose methotrexate. , 1998, The Journal of rheumatology.

[51]  D. Scott,et al.  Churg-Strauss syndrome in a district hospital. , 1998, QJM : monthly journal of the Association of Physicians.

[52]  B. Hoen,et al.  A prospective, multicenter, randomized trial comparing steroids and pulse cyclophosphamide versus steroids and oral cyclophosphamide in the treatment of generalized Wegener's granulomatosis. , 1997, Arthritis and rheumatism.

[53]  J. Feehally,et al.  Age-Related Variations in Presentation and Outcome in Wegener's Granulomatosis , 1997, Journal of the Royal College of Physicians of London.

[54]  D. Adu,et al.  Controlled trial of pulse versus continuous prednisolone and cyclophosphamide in the treatment of systemic vasculitis. , 1997, QJM : monthly journal of the Association of Physicians.

[55]  C. Gordon,et al.  Damage occurs early in systemic vasculitis and is an index of outcome. , 1997, QJM : monthly journal of the Association of Physicians.

[56]  G. Kitas,et al.  Disease assessment and management of the vasculitides. , 1997, Bailliere's clinical rheumatology.

[57]  C. Gordon,et al.  Development and initial validation of the Vasculitis Damage Index for the standardized clinical assessment of damage in the systemic vasculitides. , 1997, Arthritis and rheumatism.

[58]  C. Kallenberg,et al.  Trimethoprim-sulfamethoxazole (co-trimoxazole) for the prevention of relapses of Wegener's granulomatosis , 1996 .

[59]  M. Heller,et al.  Response to trimethoprim/sulfamethoxazole in Wegener's granulomatosis depends on the phase of disease. , 1996, QJM : monthly journal of the Association of Physicians.

[60]  O. Lortholary,et al.  Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. , 1996, Medicine.

[61]  Guy Hoffman,et al.  An analysis of forty-two Wegener's granulomatosis patients treated with methotrexate and prednisone. , 1995, Arthritis and rheumatism.

[62]  C. Pusey,et al.  ANCA and predicting relapse in systemic vasculitis. , 1995, QJM : monthly journal of the Association of Physicians.

[63]  R. Moots,et al.  Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis. , 1994, QJM : monthly journal of the Association of Physicians.

[64]  M. Heller,et al.  Influence of disease manifestation and antineutrophil cytoplasmic antibody titer on the response to pulse cyclophosphamide therapy in patients with Wegener's granulomatosis. , 1994, Arthritis and rheumatism.

[65]  D. Scott,et al.  Classical versus non-renal Wegener's granulomatosis. , 1994, The Quarterly journal of medicine.

[66]  R J Falk,et al.  Nomenclature of systemic vasculitides. Proposal of an international consensus conference. , 1994, Arthritis and rheumatism.

[67]  C. Kallenberg,et al.  Association of Chronic Nasal Carriage of Staphylococcus aureus and Higher Relapse Rates in Wegener Granulomatosis , 1994, Annals of Internal Medicine.

[68]  R. Kettritz,et al.  Prognostic factors in Wegener's granulomatosis. , 1993, Postgraduate medical journal.

[69]  Guy Hoffman,et al.  The treatment of Wegener's granulomatosis with glucocorticoids and methotrexate. , 1992, Arthritis and rheumatism.

[70]  A. Douglas,et al.  Wegener's granuloma. A series of 265 British cases seen between 1975 and 1985. A report by a sub-committee of the British Thoracic Society Research Committee. , 1992, The Quarterly journal of medicine.

[71]  Guy Hoffman,et al.  Wegener Granulomatosis: An Analysis of 158 Patients , 1992, Annals of Internal Medicine.

[72]  A. Fauci,et al.  Less common manifestations and presentations of Wegener's granulomatosis. , 1992, Current opinion in rheumatology.

[73]  P. Souteyrand,et al.  Drug-induced vasculitides. , 1991, Bailliere's clinical rheumatology.

[74]  A. Flint,et al.  Diagnostic usefulness of nasal biopsy in Wegener's granulomatosis. , 1991, Human pathology.

[75]  Guy Hoffman,et al.  Interpretation of Head and Neck Biopsies in Wegener's Granulomatosis: A Pathologic Study of 126 Biopsies in 70 Patients , 1990, The American journal of surgical pathology.

[76]  B. Wechsler,et al.  [Evolutive and prognostic aspects of Wegener's granulomatosis]. , 1989, Revue du rhumatisme et des maladies osteo-articulaires.

[77]  A. Fauci,et al.  Polyangiitis overlap syndrome: Classification and prospective clinical experience , 1986 .

[78]  A. Fauci,et al.  Classification and Prospective Clinical Experience , 1986 .

[79]  M. Lockshin,et al.  Vasculitis in association with Australia antigen. , 1971, The Journal of experimental medicine.

[80]  E W WALTON,et al.  Giant-cell Granuloma of the Respiratory Tract (Wegener's Granulomatosis) , 1958, British medical journal.

[81]  J. J. Bunim,et al.  Arteritis of striated muscle in rheumatoid arthritis. , 1951, The American journal of pathology.