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Evaluation of the ACR/EULAR 2022 criteria for classification of ANCA-associated vasculitis in a population-based cohort from Sweden

Rathmann, Jens LU orcid ; Segelmark, Mårten LU and Mohammad, Aladdin LU (2023) In Rheumatology (Oxford, England) p.1-8
Abstract
Objective
To evaluate the ACR/EULAR 2022 criteria for ANCA-associated vasculitides (AAV) classification and compare them with the European Medicines Agency (EMA) algorithm and with classification based only on ANCA serology.

Methods
In the analysis, 374 cases (47% female) were classified according to the EMA algorithm, ANCA serology and ACR/EULAR criteria. The agreement rate was calculated using the kappa (κ) statistic.

Results
Under EMA, 192 patients were classified as granulomatosis with polyangiitis (GPA), 159 as microscopic polyangiitis (MPA) and 23 as eosinophilic granulomatosis with polyangiitis (EGPA). The ACR/EULAR criteria classified 199 patients as GPA, 136 as MPA and 22 as EGPA. Four patients (1.1%)... (More)
Objective
To evaluate the ACR/EULAR 2022 criteria for ANCA-associated vasculitides (AAV) classification and compare them with the European Medicines Agency (EMA) algorithm and with classification based only on ANCA serology.

Methods
In the analysis, 374 cases (47% female) were classified according to the EMA algorithm, ANCA serology and ACR/EULAR criteria. The agreement rate was calculated using the kappa (κ) statistic.

Results
Under EMA, 192 patients were classified as granulomatosis with polyangiitis (GPA), 159 as microscopic polyangiitis (MPA) and 23 as eosinophilic granulomatosis with polyangiitis (EGPA). The ACR/EULAR criteria classified 199 patients as GPA, 136 as MPA and 22 as EGPA. Four patients (1.1%) met criteria of two disease categories, and 13 (3.5%) were unclassifiable. The observed agreement between EMA and ACR/EULAR was 85% for GPA, 75% for MPA and 96% for EGPA. The unweighted κ statistic was 0.66 (95% CI: 0.60, 0.74). Of the 188 PR3-ANCA positive patients, 186 (98.9%) were classified as GPA using ACR/EULAR criteria, and 135 of 161 (83.9%) MPO-ANCA positive patients were classified as MPA. With a classification solely based on ANCA specificity, agreement with ACR/EULAR was 99% for GPA and 88% for MPA.

Conclusions
EMA and ACR/EULAR classification give similar results. A small proportion of patients cannot be classified or fall into two categories. Some patients exhibiting granuloma, a key feature of GPA, are nevertheless classified as MPA, conflicting with the current view of histopathology of AAV. There is high agreement of ANCA-based classification with that of ACR/EULAR, reflected in the considerable weight granted to ANCA in the new criteria. These crucial elements within the new criteria necessitate a consensus discussion among field experts. (Less)
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author
; and
organization
publishing date
type
Contribution to journal
publication status
epub
subject
in
Rheumatology (Oxford, England)
pages
1 - 8
publisher
Oxford University Press
external identifiers
  • pmid:37758240
ISSN
1462-0332
DOI
10.1093/rheumatology/kead516
language
English
LU publication?
yes
id
0f2e657b-3b70-494a-940f-143c25259d7b
date added to LUP
2023-11-09 16:18:48
date last changed
2023-11-10 07:06:06
@article{0f2e657b-3b70-494a-940f-143c25259d7b,
  abstract     = {{Objective<br/>To evaluate the ACR/EULAR 2022 criteria for ANCA-associated vasculitides (AAV) classification and compare them with the European Medicines Agency (EMA) algorithm and with classification based only on ANCA serology.<br/><br/>Methods<br/>In the analysis, 374 cases (47% female) were classified according to the EMA algorithm, ANCA serology and ACR/EULAR criteria. The agreement rate was calculated using the kappa (κ) statistic.<br/><br/>Results<br/>Under EMA, 192 patients were classified as granulomatosis with polyangiitis (GPA), 159 as microscopic polyangiitis (MPA) and 23 as eosinophilic granulomatosis with polyangiitis (EGPA). The ACR/EULAR criteria classified 199 patients as GPA, 136 as MPA and 22 as EGPA. Four patients (1.1%) met criteria of two disease categories, and 13 (3.5%) were unclassifiable. The observed agreement between EMA and ACR/EULAR was 85% for GPA, 75% for MPA and 96% for EGPA. The unweighted κ statistic was 0.66 (95% CI: 0.60, 0.74). Of the 188 PR3-ANCA positive patients, 186 (98.9%) were classified as GPA using ACR/EULAR criteria, and 135 of 161 (83.9%) MPO-ANCA positive patients were classified as MPA. With a classification solely based on ANCA specificity, agreement with ACR/EULAR was 99% for GPA and 88% for MPA.<br/><br/>Conclusions<br/>EMA and ACR/EULAR classification give similar results. A small proportion of patients cannot be classified or fall into two categories. Some patients exhibiting granuloma, a key feature of GPA, are nevertheless classified as MPA, conflicting with the current view of histopathology of AAV. There is high agreement of ANCA-based classification with that of ACR/EULAR, reflected in the considerable weight granted to ANCA in the new criteria. These crucial elements within the new criteria necessitate a consensus discussion among field experts.}},
  author       = {{Rathmann, Jens and Segelmark, Mårten and Mohammad, Aladdin}},
  issn         = {{1462-0332}},
  language     = {{eng}},
  pages        = {{1--8}},
  publisher    = {{Oxford University Press}},
  series       = {{Rheumatology (Oxford, England)}},
  title        = {{Evaluation of the ACR/EULAR 2022 criteria for classification of ANCA-associated vasculitis in a population-based cohort from Sweden}},
  url          = {{http://dx.doi.org/10.1093/rheumatology/kead516}},
  doi          = {{10.1093/rheumatology/kead516}},
  year         = {{2023}},
}