Vasculitis & polymyalgia rheumatica

Data-driven subclassification of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis: model-based clustering of a federated international cohort

Lancet RheumatolPublished Retrospective cohort study

Authors' conclusion

Our study reinforces the view that ANCA-associated vasculitis is not merely a binary construct. Data-driven subclassification of ANCA-associated vasculitis exhibits higher predictive value than current approaches for key outcomes.

Study context

Population
Patients diagnosed with AAV (granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA)) between Nov 1, 1966, and March 1, 2023, in six European vasculitis registries (FAIRVASC)
Participants
3,868 patients6 registries
Exposure
Data-driven cluster subclassification vs classification by clinical diagnosis (GPA/MPA) or ANCA specificity
  • SKn = 555
  • MPO-Kn = 782
  • PR3-Kn = 683
  • IMSn = 1,202
  • YRn = 646
Outcome
Identification of phenotypically distinct subgroups for a data-driven subclassification of AAV
Methodological detailsmulticentre (six European vasculitis registries), federated international cohort, model-based clustering of 17 mixed-type clinical variables, People with lived experience were involved throughout the project
  • Multicentre (six European vasculitis registries)
  • Federated international cohort
  • Model-based clustering of 17 mixed-type clinical variables
  • People with lived experience were involved throughout the project

Main finding

As reported in the abstract

Clusters identified (share of 3868 patients)
Five clusters with distinct phenotype, biochemical presentation and disease outcome
5

Observational data: associations, not effects.

Further findings

Cluster-assigned models showed improved predictive power for both patient and kidney survival vs models fitted with clinical diagnosis or ANCA status

Original publication

Data-driven subclassification of ANCA-associated vasculitis: model-based clustering of a federated international cohort. Lancet Rheumatol. Published 22 August 2024. doi:10.1016/s2665-9913(24)00187-5

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