Vasculitis & polymyalgia rheumatica

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

Rheumatology (Oxford)Published Cross-sectional study

Authors' conclusion

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.

Study context

Population
Population-based cohort of ANCA-associated vasculitides (AAV) cases, Sweden47% female
Participants
374 participants
Exposure
ACR/EULAR 2022 AAV classification criteria vs European Medicines Agency (EMA) algorithm vs ANCA serology-based classification
Outcome
Agreement between ACR/EULAR 2022 criteria and EMA algorithm (kappa statistic)
Methodological detailspopulation-based cohort, agreement analysis (kappa statistic)
  • Population-based cohort
  • Agreement analysis (kappa statistic)

Main finding

As reported in the abstract

GroupGranulomatosis with polyangiitis (GPA)Microscopic polyangiitis (MPA)Eosinophilic granulomatosis with polyangiitis (EGPA)
EMA19215923
ACR/EULAR19913622

Granulomatosis with polyangiitis (GPA)

EMA192
ACR/EULAR199

Microscopic polyangiitis (MPA)

EMA159
ACR/EULAR136

Eosinophilic granulomatosis with polyangiitis (EGPA)

EMA23
ACR/EULAR22
Observed agreement EMA vs ACR/EULAR, GPA
85%
Observed agreement EMA vs ACR/EULAR, MPA
75%
Observed agreement EMA vs ACR/EULAR, EGPA
96%
Unweighted κ statistic
0.66 (95% CI: 0.60, 0.74)

Observational data: associations, not effects.

Further findings

ACR/EULAR classification by ANCA specificity

PR3-ANCA positive (n=188) classified as GPA
186 (98.9%)
MPO-ANCA positive classified as MPA
135 of 161 (83.9%)

Agreement of ANCA serology-based classification with ACR/EULAR

GPA
99%
MPA
88%

Original publication

Evaluation of the ACR/EULAR 2022 criteria for classification of ANCA-associated vasculitis in a population-based cohort from Sweden. Rheumatology (Oxford). Published 27 September 2023. doi:10.1093/rheumatology/kead516

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