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BVAS version 3 and BVAS/GPA: standing on the same line?

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Abstract

Introduction/objectives

Birmingham vasculitis activity score (BVAS) version 3 (BVAS 3.0) and BVAS/granulomatosis with polyangiitis (BVAS/GPA) are used as indicators of disease activity in anti-neutrophil cytoplasmic antibody-associated vasculitis. We evaluated the association between these indices and the significance in patients with GPA and microscopic polyangiitis (GPA/MPA).

Methods

We retrospectively reviewed the records of 203 patients with GPA/MPA in our hospital. The correlation between BVAS 3.0 and BVAS/GPA with the five-factor score (FFS) and laboratory data was investigated. The episodes of all-cause mortality, end-stage renal disease, and disease relapse were counted as adverse clinical outcomes. Multivariate Cox hazard analyses were performed to assess the relationships between both indices and patient outcomes.

Results

Sixty-five (32.0%) and 138 (68.0%) patients with GPA and MPA were included. The median BVAS 3.0 was significantly higher in patients with MPA than in those with GPA (13.0 vs. 11.0, p = 0.015), whereas BVAS/GPA was higher in patients with GPA (4.0 vs. 3.0, p = 0.001). BVAS 3.0 and BVAS/GPA correlated significantly (r = 0.670, p < 0.001); both BVAS 3.0 and BVAS/GPA were shown to be associated with the outcomes investigated in separate Cox models. However, the correlation between BVAS 3.0 and BVAS/GPA was especially higher in a subgroup of patients with MPA than in those with GPA (MPA: r = 0.817, p < 0.001 vs. GPA: r = 0.570, p < 0.001) and with renal involvement (r = 0.676, p < 0.001).

Conclusions

Although both BVAS 3.0 and BVAS/GPA significantly correlated and predicted outcomes well in those with GPA/MPA, a discord was observed based on disease subtypes and organ involvement.

Key Points

• BVAS 3.0 and BVAS/GPA significantly correlated and predicted outcomes in those with GPA/MPA.

• A discordance was also observed based on disease subtypes and organ involvement.

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Data availability

The datasets used and/or analysed during the current study are available from the corresponding author on reasonable request.

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Funding

This research was supported by a grant from the Korea Health Technology R&D Project through the Korea Health Industry Development Institute, funded by the Ministry of Health and Welfare (HI14C1324), the Handok Inc., Seoul, Republic of Korea (HANDOK 2021-006), and CELLTRION PHARM, Inc. Chungcheongbuk-do, Republic of Korea (NCR 2019-6).

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Author notes

  1. Sung Soo Ahn and Jang Woo Ha contributed equally to this work.

Authors and Affiliations

  1. Department of Internal Medicine, Yongin Severance Hospital, Yonsei University College of Medicine, Yongin, Republic of Korea

    Sung Soo Ahn

  2. Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun–gu, Seoul, 03722, Republic of Korea

    Jang Woo Ha, Yong-Beom Park & Sang-Won Lee

  3. Institute for Immunology and Immunological Diseases, Yonsei University College of Medicine, Seoul, Republic of Korea

    Yong-Beom Park & Sang-Won Lee

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Contributions

Conceptualisation, S.S.A. and S-W.L.; methodology, S.S.A. and J.W.H.; software, S.S.A.; validation, S.S.A. and J.W.H.; formal analysis, S.S.A. and J.W.H.; investigation, S.S.A.; resources, S.S.A., J.W.H., Y-B.P., and S-W.L.; data curation, S.S.A. and J.W.H.; writing—original draft preparation, S.S.A. and J.W.H.; writing—review and editing, S.S.A., J.W.H., Y-B.P., and S-W.L.; visualisation, S.S.A. and J.W.H.; supervision, Y-B.P. and S-W.L.; project administration, S.S.A., J.W.H., and S-W.L. All authors have read and agreed to the published version of the manuscript.

Corresponding author

Correspondence to Sang-Won Lee.

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Supplementary Fig. 1

Flowchart describing the inclusion and exclusion of patients. Among the 258 patients with the diagnosis of GPA/MPA searched through the hospital’s electronic medical review system, a total of 55 patients were excluded, reaching the number of 203. GPA/MPA: Granulomatosis with polyangiitis and microscopic polyangiitis; ACR: American College of Rheumatology; EMA: European Medicines Agency; CHCC: Chapel Hill Consensus Conference; BVAS: Birmingham vasculitis activity score; FFS: Five-factor score. (PNG 117 kb)

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Ahn, S.S., Ha, J.W., Park, YB. et al. BVAS version 3 and BVAS/GPA: standing on the same line?. Clin Rheumatol 41, 3429–3437 (2022). https://doi.org/10.1007/s10067-022-06267-z

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  • DOI: https://doi.org/10.1007/s10067-022-06267-z

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