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

• Type: Article in International Scientific Journal
• Date: 2022

Authors

Suppiah, R (Author) Other; Robson, JC (Author) Other; Grayson, PC (Author) Other; Ponte, C (Author) Other; Craven, A (Author) Other; Khalid, S (Author) Other; Judge, A (Author) Other; Hutchings, A (Author) Other; Merkel, PA (Author) Other; Luqmani, RA (Author) Other; Watts, RA (Author) Other; Elsa Azevedo (Author) FMUP

Journal

Title: Annals of the Rheumatic Diseases

Vol. 81

Pages: 321-326

ISSN: 0003-4967

Publisher: BMJ PUBLISHING GROUP

Indexing

Scopus - 138 Citations

Other information

Authenticus ID: P-00W-6P3

DOI: 10.1136/annrheumdis-2021-221796

Abstract (EN): 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: (1) identification of candidate items using consensus methodology, (2) prospective collection of candidate items present at the time of diagnosis, (3) data-driven reduction of the number of candidate items, (4) expert panel review of cases to define the reference diagnosis and (5) derivation of a points-based risk score for disease classification in a development set using least absolute shrinkage and selection operator 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 408 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 final criteria and their weights were as follows: perinuclear antineutrophil cytoplasmic antibody (ANCA) or anti-myeloperoxidase-ANCA positivity (+6), pauci-immune glomerulonephritis (+3), lung fibrosis or interstitial lung disease (+3), sino-nasal symptoms or signs (-3), cytoplasmic ANCA or anti-proteinase 3 ANCA positivity (-1) and eosinophil count ¿1×109/L (-4). After excluding mimics of vasculitis, a patient with a diagnosis of small- or medium-vessel vasculitis could be classified as having MPA with a cumulative score of ¿5 points. When these criteria were tested in the validation data set, the sensitivity was 91% (95% CI 85% to 95%) and the specificity was 94% (95% CI 92% to 96%). CONCLUSION: The 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for MPA are now validated for use in clinical research. © Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.

Language: English

Type (Professor's evaluation): Scientific