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2022 American College of Rheumatology/European Alliance of Associations for rheumatology classification criteria for microscopic polyangiitis

Suppiah, Ravi; Robson, Jo; Grayson, Peter; Ponte, Cristina; Craven, Anthea; Khalid, Sara; Judge, Andrew; Hutchings, Andrew; Merkel, Peter; Luqmani, Raashid; Watts, Richard

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Authors

Ravi Suppiah

Jo Robson Jo.Robson@uwe.ac.uk
Consultant Associate Professor in Rheumatology

Peter Grayson

Cristina Ponte

Anthea Craven

Sara Khalid

Andrew Judge

Andrew Hutchings

Peter Merkel

Raashid Luqmani

Richard Watts



Abstract

ABSTRACT
Objective: To develop and validate classification criteria for microscopic polyangiitis (MPA).
Methods: Patients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in five phases: i) Identification of candidate items using consensus methodology; ii) Prospective data collection of candidate items present at the time of diagnosis; iii) Data-driven reduction of candidate items; iv) Expert panel review of cases to define the reference diagnosis; v) Derivation of a points-based risk score for disease classification in a development set using lasso logistic regression with subsequent validation of performance characteristics in an independent set of cases and comparators.
Results: The development set for MPA consisted of 149 cases of MPA and 409 comparators. The validation set consisted of an additional 142 cases of MPA and 414 comparators. From 91 candidate items, regression analysis identified 10 items for MPA, 6 of which were retained. The weighting of final criteria items was: i) pANCA or anti-MPO ANCA positivity (+6), ii) Pauci-immune glomerulonephritis (+3), iii) Lung fibrosis or interstitial lung disease (+3), iv) Sino-nasal symptoms or signs (-3), v) cANCA or anti-PR3 ANCA positivity (-1), and vi) Eosinophil count ≥ 1x109/L (-4). After excluding mimics of vasculitis, a patient with a diagnosis of small- or medium-vessel vasculitis could be classified as MPA with a cumulative score of ≥ 5 points. When these criteria were tested in the validation dataset, the sensitivity was 91% (95% confidence interval [95% CI] 85-95%) and the specificity was 94% (95% CI 92-96%).
Conclusion: The 2021 ACR-EULAR MPA Classification Criteria are now validated for use in clinical research.

Citation

Suppiah, R., Robson, J., Grayson, P., Ponte, C., Craven, A., Khalid, S., …Watts, R. (2022). 2022 American College of Rheumatology/European Alliance of Associations for rheumatology classification criteria for microscopic polyangiitis. Arthritis and Rheumatology, 74(3), 400-406. https://doi.org/10.1002/art.41983

Journal Article Type Article
Acceptance Date Sep 21, 2021
Online Publication Date Feb 2, 2022
Publication Date Mar 1, 2022
Deposit Date Jun 2, 2021
Publicly Available Date Feb 3, 2023
Journal Arthritis and Rheumatology
Print ISSN 2326-5191
Electronic ISSN 2326-5205
Publisher Wiley
Peer Reviewed Peer Reviewed
Volume 74
Issue 3
Pages 400-406
DOI https://doi.org/10.1002/art.41983
Public URL https://uwe-repository.worktribe.com/output/7438074

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This is the peer reviewed version of the following article:Suppiah, R., Robson, J., Grayson, P., Ponte, C., Craven, A., Khalid, S., …Watts, R. (2022). 2022 American College of Rheumatology/European Alliance of Associations for rheumatology classification criteria for microscopic polyangiitis. Arthritis and Rheumatology, 74(3), 400-406, which has been published in final form at https://doi.org/10.1002/art.41983. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Use of Self-Archived Versions



2022 American College of Rheumatology/European Alliance of Associations for rheumatology classification criteria for microscopic polyangiitis (459 Kb)
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http://www.rioxx.net/licenses/all-rights-reserved

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http://www.rioxx.net/licenses/all-rights-reserved

Copyright Statement
This is the peer reviewed version of the following article: Suppiah, R., Robson, J., Grayson, P., Ponte, C., Craven, A., Khalid, S., …Watts, R. (2022). 2022 American College of Rheumatology/European Alliance of Associations for rheumatology classification criteria for microscopic polyangiitis. Arthritis and Rheumatology, 74(3), 400-406, which has been published in final form at https://doi.org/10.1002/art.41983. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Use of Self-Archived Versions




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