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We performed multiple correspondence and cluster analysis regarding 427 clinically diagnosed AAV patients excluding eosinophilic granulomatosis with polyangiitis. Model 1 included the ANCA phenotype, items of the Birmingham Vasculitis Activity Score, and interstitial lung disease; model 2 included serum creatinine (s-Cr) and C-reactive protein (CRP) levels with model 1 components. In seven clusters determined in model 1, the ANCA-negative (n\u2009=\u20098) and proteinase 3-ANCA-positive (n\u2009=\u200941) groups emerged as two distinct clusters. The other five myeloperoxidase-ANCA-positive clusters were characterized by ear, nose, and throat (ENT) (n\u2009=\u200947); cutaneous (n\u2009=\u200936); renal (n\u2009=\u2009256), non-renal (n\u2009=\u200933); and both ENT and cutaneous symptoms (n\u2009=\u20096). Four clusters in model 2 were characterized by myeloperoxidase-ANCA negativity (n\u2009=\u200942), without s-Cr elevation (&lt;\u20091.3\u00a0mg\/dL) (n\u2009=\u2009157), s-Cr elevation (\u2265\u20091.3\u00a0mg\/dL) with high CRP (&gt;\u200910\u00a0mg\/dL) (n\u2009=\u200971), or s-Cr elevation (\u2265\u20091.3\u00a0mg\/dL) without high CRP (\u2264\u200910\u00a0mg\/dL) (n\u2009=\u2009157). Overall, renal, and relapse-free survival rates were significantly different across the four clusters in model 2. ENT, cutaneous, and renal symptoms may be useful in characterization of Japanese AAV patients with myeloperoxidase-ANCA. The combination of s-Cr and CRP levels may be predictive of prognosis.<\/jats:p>","DOI":"10.1038\/s41598-021-84627-6","type":"journal-article","created":{"date-parts":[[2021,3,4]],"date-time":"2021-03-04T11:04:29Z","timestamp":1614855869000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Exploratory classification of clinical phenotypes in Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis using cluster analysis"],"prefix":"10.1038","volume":"11","author":[{"given":"Haruki","family":"Watanabe","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ken-ei","family":"Sada","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masayoshi","family":"Harigai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koichi","family":"Amano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroaki","family":"Dobashi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshinari","family":"Takasaki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shouichi","family":"Fujimoto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tatsuya","family":"Atsumi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kunihiro","family":"Yamagata","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sakae","family":"Homma","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshihiro","family":"Arimura","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hirofumi","family":"Makino","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"Research Committee of Intractable Vasculitis Syndrome (JPVAS)","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"Research Committee of Intractable Renal Disease of the Ministry of Health, Labour, and Welfare of Japan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,3,4]]},"reference":[{"issue":"1","key":"84627_CR1","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1002\/art.37715","volume":"65","author":"J Jennette","year":"2013","unstructured":"Jennette, J. et al. 2012 revised international Chapel Hill consensus conference nomenclature of vasculitides. 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MH has received unrestricted research grants from AbbVie Japan GK, Ayumi Pharmaceutical Co.; Bristol Myers Squibb Co., Ltd.; Eisai Co., Ltd., Nippon Kayaku Co., Ltd.; Mitsubishi Tanabe Pharma Co.; and Teijin Pharma Ltd. MH has received speaker\u2019s fee from Boehringer-ingelheim, Kissei Pharmaceutical Co., Ltd.; Eli Lilly Japan K.K.; Chugai Pharmaceutical Co., Ltd. MH is a consultant for AbbVie, Boehringer-ingelheim, Kissei Pharmaceutical Co., Ltd. and Teijin Pharma. HM is a consultant for AbbVie, Boehringer-Ingelheim and Teijin. The other authors declare no competing interests.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"5223"}}