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

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

Authors

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

Peter Grayson

Cristina Ponte

Ravi Suppiah

Anthea Craven

Andrew Judge

Sara Khalid

Andrew Hutchings

Richard Watts

Peter Merkel

Raashid Luqmani



Abstract

Objective: To develop and validate revised classification criteria for granulomatosis with polyangiitis (GPA).
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 collection of candidate items present at time of diagnosis; iii) Data-driven reduction of candidate items; iv) Expert review to define the reference diagnosis; and 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 GPA consisted of 578 GPA cases and 652 comparators. The validation set consisted of an additional 146 cases of GPA and 161 comparators. From 91 candidate items, regression analysis identified 26 items for GPA, 10 of which were retained. The weighting of final criteria items was: i) Bloody nasal discharge, nasal crusting, or sino-nasal congestion (+3), ii) Cartilaginous involvement (+2), iii) Conductive or sensorineural hearing loss (+1), iv) cANCA or anti-PR3 ANCA positivity (+5), v) Pulmonary nodules, mass, or cavitation on chest imaging (+2), vi) Granuloma or giant cells on biopsy (+2), vii) Inflammation or consolidation of the nasal/paranasal sinuses on imaging (+1), viii) Pauci-immune glomerulonephritis (+1), ix) pANCA or anti-MPO ANCA positivity (-1), and x) Eosinophil count ≥ 1 (x109/L) (-4). After excluding mimics of vasculitis, a patient with a diagnosis of small- or medium-vessel vasculitis could be classified as GPA with a cumulative score of ≥ 5 points. When these criteria were tested in the validation dataset, the sensitivity was 93% (95% confidence interval [95% CI] 87-96%) and the specificity was 94% (95% CI 89-97%).
Conclusion: The 2021 ACR-EULAR GPA Classification Criteria demonstrate strong performance characteristics and are validated for use in research.

Citation

Robson, J., Grayson, P., Ponte, C., Suppiah, R., Craven, A., Judge, A., …Luqmani, R. (2022). 2022 American College of Rheumatology/European Alliance of Associations for rheumatology classification criteria for granulomatosis with polyangiitis. Arthritis and Rheumatology, 74(3), 393-399. https://doi.org/10.1002/art.41986

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 Mar 28, 2024
Journal Arthritis and Rheumatology
Print ISSN 2326-5191
Electronic ISSN 2326-5205
Publisher Wiley
Peer Reviewed Peer Reviewed
Volume 74
Issue 3
Pages 393-399
DOI https://doi.org/10.1002/art.41986
Public URL https://uwe-repository.worktribe.com/output/7438036

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This is the peer reviewed version of the following article: This is the peer reviewed version of the following article: Robson, J., Grayson, P., Ponte, C., Suppiah, R., Craven, A., Judge, A., …Luqmani, R. (2022). 2022 American College of Rheumatology/European Alliance of Associations for rheumatology classification criteria for granulomatosis with polyangiitis. Arthritis and Rheumatology, 74(3), 393-399, which has been published in final form at https://doi.org/10.1002/art.41986. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Use of Self-Archived Versions. which has been published in final form at https://doi.org/10.1002/art.41986. 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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Copyright Statement
This is the peer reviewed version of the following article: Robson, J., Grayson, P., Ponte, C., Suppiah, R., Craven, A., Judge, A., …Luqmani, R. (2022). 2022 American College of Rheumatology/European Alliance of Associations for rheumatology classification criteria for granulomatosis with polyangiitis. Arthritis and Rheumatology, 74(3), 393-399, which has been published in final form at https://doi.org/10.1002/art.41986. 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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