Vasculitis & polymyalgia rheumatica
Clinical phenotypes of ANCA-associated vasculitis using ensemble clustering: prognostic and therapeutic insights from two Japanese cohorts
Authors' conclusion
Organ involvement-based ensemble clustering identifies clinically meaningful and reproducible AAV phenotypes with distinct prognostic and therapeutic profiles.
Study context
- Population
- Patients with newly diagnosed microscopic polyangiitis (MPA) or granulomatosis with polyangiitis (GPA) in two nationwide Japanese registries
- Participants
- 726 patients
- Exposure
- Data-driven organ-involvement phenotypes of MPA and GPA
- Outcome
- Data-driven clinical phenotypes based on patterns of organ involvement
Methodological detailsmulticentre, two nationwide Japanese registries (J-CANVAS derivation; JPVAS validation), ensemble clustering, classification and regression tree (CART) replication
- Multicentre
- Two nationwide Japanese registries (J-CANVAS derivation; JPVAS validation)
- Ensemble clustering
- CART replication
Main finding
As reported in the abstract
Clinical phenotypes identified by ensemble clustering
- Four reproducible clinical phenotypes identified: renal-dominant without interstitial lung disease (ILD); renal-dominant with ILD; systemic multi-organ; Ear, nose and throat (ENT)-dominant
- CART reproduced these clusters with good concordance
Observational data: associations, not effects.
Further findings
Overall survival and relapse incidence
- Overall survival and relapse incidence differed across clusters
- ENT-dominant: favourable survival but higher relapse risk
Relapse-free survival with RTX vs CYC in ENT-dominant cluster
- Trend towards improved relapse-free survival with RTX compared with CYC, even among MPO-ANCA-positive patients
Original publication
Clinical phenotypes of ANCA-associated vasculitis using ensemble clustering: prognostic and therapeutic insights from two Japanese cohorts. Rheumatology (Oxford). Published 17 September 2026. doi:10.
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