Clinical phenotypes of ANCA-associated vasculitis using ensemble clustering: prognostic and therapeutic insights from two Japanese cohorts.
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Inoue Hironori, Kida Takashi, Fujioka Kazuki, Omura Satoshi, Yanagida Takuya, Shimada Yuki, Kitai Junya, Seno Takahiro, Kohno Masataka, Nakagomi Daiki, Abe Yoshiyuki, Wada Makoto, Takizawa Naoho, Nomura Atsushi, Kukida Yuji, Kondo Naoya, Takagi Hirosuke, Endo Koji, Hirata Shintaro, Azuma Naoto, Takeuchi Tohru, Fukui Shoichi, Kamada Kazuro, Yanai Ryo, Matsuo Yusuke, Shimojima Yasuhiro, Nishioka Ryo, Okazaki Ryota, Takata Tomoaki, Moriyama Mayuko, Takatani Ayuko, Miyawaki Yoshia, Shirai Tsuyoshi, Dobashi Hiroaki, Ito Takafumi, Matsumoto Isao, Takada Toshihiko, Ito-Ihara Toshiko, Yajima Nobuyuki, Kawaguchi Takashi, Ikeuchi Hidekazu, Nakaya Izaya, Shimoyama Kumiko, Amano Koichi, Endo Tomomi, Ushio Yusuke, Komagata Yoshinori, Naniwa Taio, Kawakami Atsushi, Higuchi Tomoaki, Nagasaka Kenji, Uchida Haruhito A, Tamura Naoto, Kawahito Yutaka, J-CANVAS Study and JPVAS Cohort Study Collaborative Group Investigators
Аннотация
METHODS: We conducted a multicentre retrospective cohort study using two nationwide Japanese registries (J-CANVAS as derivation; JPVAS as validation). Ensemble clustering was applied to high-dimensional organ involvement data derived from the Birmingham Vasculitis Activity Score and additional clinically relevant manifestations. The resulting clusters were replicated using classification and regression tree (CART) across the derivation and validation cohorts, and associations between cluster memberships and clinical outcomes (overall survival and relapse), as well as response to induction therapy, were evaluated.
RESULTS: Among 726 patients with newly diagnosed MPA or GPA, ensemble clustering identified four reproducible clinical phenotypes: (a) renal-dominant without interstitial lung disease (ILD); (b) renal-dominant with ILD; (c) systemic multi-organ and (d) ear, nose and throat (ENT)-dominant. CART reproduced these clusters with good concordance. Overall survival and relapse incidence differed across clusters. The ENT-dominant cluster demonstrated favourable survival but a higher relapse risk and showed a trend towards improved relapse-free survival with rituximab compared with CYC, even among MPO-ANCA-positive patients.
CONCLUSION: Organ involvement-based ensemble clustering identifies clinically meaningful and reproducible AAV phenotypes with distinct prognostic and therapeutic profiles. Integrating phenotypic patterns with ANCA serotype may enhance risk stratification and support more individualized management strategies in AAV.