
URUHA Akinori
Shinshu University Hospital Intractable Disease Care Center
Senior Assistant Professor
Researcher Information
Research Keyword
- Myopathy, Myositis, Muscle pathology, Muscle biopsy, Muscle imaging, Inclusion body myositis, Sporadic late-onset nemaline myopathy, Immune-mediated necrotizing myopathy, Dermatomyositis, Antisynthetase syndrome, Overlap myositis, Myopathy with early respiratory failure
Web site
Career
- 2025 - Present
Shinshu University, School of Medicine, Department of Medicine (Neurology and Rheumatology), Senior Assistant Professor - 2020 - 2025
Tokyo Metropolitan Neurological Hospital, Department of Neurology - 2019 - 2020
Charité - Universitätsmedizin Berlin, Department of Neuropathology - 2018 - 2019
Tokyo Metropolitan Neurological Hospital, Department of Neurology - 2016 - 2018
INSERM, UPMC - Paris VI (currently Sorbonne University); Department of Internal Medicine and Clinical Immunology, Pitié-Salpêtrière University Hospital; Institute of Myology (myositis research team) - 2012 - 2016
National Center of Neurology and Psychiatry, National Institute of Neuroscience, NCNP - 2008 - 2012
Tokyo Metropolitan Neurological Hospital, Department of Neurology - 2006 - 2008
Yoka Public Hospital, Resident
Research activity information
Award
Paper
- Myositis associated with antimitochondrial autoantibodies presenting with early respiratory failure.
Kentaro Kawama; Shinsuke Tobisawa; Akinori Uruha; Satoko Uruha; Hirotake Nishimura; Hiromi Onizuka; Kenta Masui; Saeko Yoshizawa; Tomoko Yamamoto; Takashi Komori; Keisuke Ishizawa; Kazushi Takahashi
Neuromuscular disorders : NMD, 52, 105395-105395, Jul. 2025
Corresponding - Anti-Ku + myositis: an acquired inflammatory protein-aggregate myopathy.
Marie-Therese Holzer; Akinori Uruha; Andreas Roos; Andreas Hentschel; Anne Schänzer; Joachim Weis; Kristl G Claeys; Benedikt Schoser; Federica Montagnese; Hans-Hilmar Goebel; Melanie Huber; Sarah Léonard-Louis; Ina Kötter; Nathalie Streichenberger; Laure Gallay; Olivier Benveniste; Udo Schneider; Corinna Preusse; Martin Krusche; Werner Stenzel
Acta neuropathologica, 148(1), 6-6, Jul. 2024 - Pathologic Features of Anti-Ku Myositis.
Munenori Oyama; Marie-Therese Holzer; Yuko Ohnuki; Yoshihiko Saito; Yukako Nishimori; Shingo Suzuki; Takashi Shiina; Sarah Leonard-Louis; Olivier Benveniste; Udo Schneider; Werner Stenzel; Ichizo Nishino; Shigeaki Suzuki; Akinori Uruha
Neurology, 102(8), e209268, Apr. 2024
Last, Corresponding - Immunopathological features of myopathy associated with small-to-medium-sized vessel vasculitis and differences from autoimmune myositis.
Shun Nomura; Yasuhiro Shimojima; Takanori Ichikawa; Daigo Miyazaki; Akinori Uruha; Dai Kishida; Yoshiki Sekijima
Clinical and experimental rheumatology, 42(4), 786-794, Apr. 2024 - "Amyopathic" MDA5-positive dermatomyositis with severe lung involvement presenting with net myositic morphological features - insights from an autopsy study.
Benjamin Englert; Carsten Dittmayer; Hans-Hilmar Goebel; Udo Schneider; Marie-Therese Holzer; Akinori Uruha; Werner Stenzel
Neuromuscular disorders : NMD, 36, 42-47, Mar. 2024 - Association Between HLA Alleles and Autoantibodies in Dermatomyositis Defined by Sarcoplasmic Expression of Myxovirus Resistance Protein A.
Munenori Oyama; Yuko Ohnuki; Akinori Uruha; Yoshihiko Saito; Yukako Nishimori; Shingo Suzuki; Michio Inoue; Jantima Tanboon; Naoko Okiyama; Takashi Shiina; Ichizo Nishino; Shigeaki Suzuki
The Journal of rheumatology, 50(9), 1159-1164, Sep. 2023 - Morphologic and Molecular Patterns of Polymyositis With Mitochondrial Pathology and Inclusion Body Myositis.
Felix Kleefeld; Akinori Uruha; Anne Schänzer; Anna Nishimura; Andreas Roos; Udo Schneider; Hans H Goebel; Markus Schuelke; Katrin Hahn; Corinna Preusse; Werner Stenzel
Neurology, 99(20), e2212-e2222, Nov. 2022 - Skeletal muscle provides the immunological micro-milieu for specific plasma cells in anti-synthetase syndrome-associated myositis.
Corinna Preuße; Barbara Paesler; Christopher Nelke; Derya Cengiz; Thomas Müntefering; Andreas Roos; Damien Amelin; Yves Allenbach; Akinori Uruha; Carsten Dittmayer; Andreas Hentschel; Marc Pawlitzki; Sarah Hoffmann; Sara Timm; Sarah Leonard Louis; Nora F Dengler; Heinz Wiendl; Jan D Lünemann; Albert Sickmann; Baptiste Hervier; Sven G Meuth; Udo Schneider; Anne Schänzer; Sabine Krause; Stylianos Tomaras; Eugen Feist; Rebecca Hasseli; Hans-Hilmar Goebel; Laure Gallay; Nathalie Streichenberger; Olivier Benveniste; Werner Stenzel; Tobias Ruck
Acta neuropathologica, 144(2), 353-372, Aug. 2022 - Myoclonic Epilepsy with Ragged-red Fibers with Intranuclear Inclusions.
Tomoya Kawazoe; Shinsuke Tobisawa; Keizo Sugaya; Akinori Uruha; Kazuhito Miyamoto; Takashi Komori; Yu-Ichi Goto; Ichizo Nishino; Hiroshi Yoshihashi; Takeshi Mizuguchi; Naomichi Matsumoto; Naohiro Egawa; Akihiro Kawata; Eiji Isozaki
Internal medicine (Tokyo, Japan), 61(4), 547-552, Feb. 2022 - Successful plasmapheresis and immunoglobulin treatment for severe lipid storage myopathy: Doing the right thing for the wrong reason.
Felix Kleefeld; Anja von Renesse; Carsten Dittmayer; Lutz Harms; Josefine Radke; Helena Radbruch; Hans-Hilmar Goebel; Florence Pache; Udo Schneider; Markus Schuelke; Akinori Uruha; Werner Stenzel
Neuropathology and applied neurobiology, 48(1), e12731, Feb. 2022 - SIGLEC1 enables straightforward assessment of type I interferon activity in idiopathic inflammatory myopathies.
Manuel Graf; Sae Lim von Stuckrad; Akinori Uruha; Jens Klotsche; Lydia Zorn-Pauly; Nadine Unterwalder; Thomas Buttgereit; Martin Krusche; Christian Meisel; Gerd R Burmester; Falk Hiepe; Robert Biesen; Tilmann Kallinich; Werner Stenzel; Udo Schneider; Thomas Rose
RMD open, 8(1), Feb. 2022 - Mild form of Danon disease: two case reports.
Toshio Yasui; Utako Nagaoka; Yasushi Oya; Akinori Uruha; Jun Karashima; Asuka Funai; Kazuhito Miyamoto; Shiro Matsubara; Keizo Sugaya; Kazushi Takahashi; Michio Inoue; Mariko Okubo; Kazuma Sugie; Ichizo Nishino
Neuromuscular disorders : NMD, 31(11), 1207-1211, Nov. 2021
Corresponding - Systemic sclerosis-associated myositis features minimal inflammation and characteristic capillary pathology.
Elise Siegert; Akinori Uruha; Hans-Hilmar Goebel; Corinna Preuße; Vincent Casteleyn; Felix Kleefeld; Rieke Alten; Gerd R Burmester; Udo Schneider; Jakob Höppner; Kathrin Hahn; Carsten Dittmayer; Werner Stenzel
Acta neuropathologica, 141(6), 917-927, Jun. 2021
Lead - Inflammatory features in sporadic late-onset nemaline myopathy are independent from monoclonal gammopathy.
Jantima Tanboon; Akinori Uruha; Yukie Arahata; Carsten Dittmayer; Leonille Schweizer; Hans-Hilmar Goebel; Ichizo Nishino; Werner Stenzel
Brain pathology (Zurich, Switzerland), 31(3), e12962, May 2021
Lead - A novel mutation in NEB causing foetal nemaline myopathy with arthrogryposis during early gestation.
Maria L Rocha; Carsten Dittmayer; Akinori Uruha; Dirk Korinth; Rabih Chaoui; Dietmar Schlembach; Rainer Rossi; Katarina Pelin; Eun Kyung Suk; Simone Schmid; Hans H Goebel; Markus Schuelke; Werner Stenzel; Benjamin Englert
Neuromuscular disorders : NMD, 31(3), 239-245, Mar. 2021 - Sirolimus for treatment of patients with inclusion body myositis: a randomised, double-blind, placebo-controlled, proof-of-concept, phase 2b trial.
Olivier Benveniste; Jean-Yves Hogrel; Lisa Belin; Mélanie Annoussamy; Damien Bachasson; Aude Rigolet; Pascal Laforet; Gaëlle Dzangué-Tchoupou; Joe-Elie Salem; Lee S Nguyen; Tanya Stojkovic; Noel Zahr; Baptiste Hervier; Océane Landon-Cardinal; Anthony Behin; Edith Guilloux; Harmen Reyngoudt; Damien Amelin; Akinori Uruha; Kuberaka Mariampillai; Benjamin Marty; Bruno Eymard; Jean-Sébastien Hulot; Steven A Greenberg; Pierre G Carlier; Yves Allenbach
The Lancet. Rheumatology, 3(1), e40-e48, Jan. 2021 - Myositis with sarcoplasmic inclusions in Nakajo-Nishimura syndrome: a genetic inflammatory myopathy.
T Ayaki; K Murata; N Kanazawa; A Uruha; K Ohmura; K Sugie; S Kasagi; F Li; M Mori; R Nakajima; T Sasai; I Nishino; S Ueno; M Urushitani; F Furukawa; H Ito; R Takahashi
Neuropathology and applied neurobiology, 46(6), 579-587, Oct. 2020 - Rimmed Vacuoles in Myositis Associated with Antimitochondrial Antibody.
Rui Shimazaki; Akinori Uruha; Hideki Kimura; Utako Nagaoka; Tomoya Kawazoe; Satoshi Yamashita; Takashi Komori; Kazuhito Miyamoto; Shiro Matsubara; Keizo Sugaya; Masahiro Nagao; Eiji Isozaki
Journal of clinical neurology (Seoul, Korea), 16(3), 510-512, Jul. 2020 - Anti-RNP antibodies delineate a subgroup of myositis: A systematic retrospective study on 46 patients.
Nadège Wesner; Akinori Uruha; Shigeaki Suzuki; Kubéraka Mariampillai; Benjamin Granger; Nicolas Champtiaux; Aude Rigolet; Yoland Schoindre; Sylvain Lejeune; Perrine Guillaume-Jugnot; Matthieu Vautier; Baptiste Hervier; Anne Simon; Françoise Granier; Laure Gallay; Ichizo Nishino; Olivier Benveniste; Yves Allenbach
Autoimmunity reviews, 19(3), 102465-102465, Mar. 2020
Lead - A 67-Year-Old Man with Leg Weakness and Hypertrophic Cardiomyopathy.
Tomoya Kawazoe; Akinori Uruha; Madoka Mori-Yoshimura; Yuko Saito; Shu-Ichi Ikeda; Mike Saji; Akira Tanimura; Tomofumi Amano; Tomoko Okamoto; Yasushi Oya; Miho Murata; Ichizo Nishino; Yuji Takahashi
Brain pathology (Zurich, Switzerland), 30(2), 427-428, Mar. 2020 - HLA-DRB1 allele and autoantibody profiles in Japanese patients with inclusion body myositis.
Munenori Oyama; Yuko Ohnuki; Michio Inoue; Akinori Uruha; Satoshi Yamashita; Sachiko Yutani; Jantima Tanboon; Jin Nakahara; Shingo Suzuki; Takashi Shiina; Ichizo Nishino; Shigeaki Suzuki
PloS one, 15(8), e0237890, 2020 - Diagnostic potential of sarcoplasmic myxovirus resistance protein A expression in subsets of dermatomyositis.
A Uruha; Y Allenbach; J-L Charuel; L Musset; A Aussy; O Boyer; K Mariampillai; O Landon-Cardinal; C Rasmussen; L Bolko; T Maisonobe; S Leonard-Louis; S Suzuki; I Nishino; W Stenzel; O Benveniste
Neuropathology and applied neurobiology, 45(5), 513-522, Aug. 2019
Lead - Absence of sarcoplasmic myxovirus resistance protein A (MxA) expression in antisynthetase syndrome in a cohort of 194 cases.
M Inoue; J Tanboon; M Okubo; K Theerawat; Y Saito; M Ogasawara; L A Indrawati; A Uruha; N Okiyama; M Fujimoto; S Suzuki; I Nishino
Neuropathology and applied neurobiology, 45(5), 523-524, Aug. 2019 - Inflammatory myopathy associated with PD-1 inhibitors.
Morinobu Seki; Akinori Uruha; Yuko Ohnuki; Sachiko Kamada; Tomoko Noda; Asako Onda; Masayuki Ohira; Aiko Isami; Sumie Hiramatsu; Makoto Hibino; Shunya Nakane; Seiya Noda; Sachiko Yutani; Akira Hanazono; Hiroshi Yaguchi; Masaki Takao; Takashi Shiina; Masahisa Katsuno; Jin Nakahara; Shiro Matsubara; Ichizo Nishino; Shigeaki Suzuki
Journal of autoimmunity, 100, 105-113, Jun. 2019 - Pediatric necrotizing myopathy associated with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase antibodies.
Wen-Chen Liang; Akinori Uruha; Shigeaki Suzuki; Nobuyuki Murakami; Eri Takeshita; Wan-Zi Chen; Yuh-Jyh Jong; Yukari Endo; Hirofumi Komaki; Tatsuya Fujii; Yutaka Kawano; Madoka Mori-Yoshimura; Yasushi Oya; Jianying Xi; Wenhua Zhu; Chongbo Zhao; Yurika Watanabe; Keisuke Ikemoto; Atsuko Nishikawa; Kohei Hamanaka; Satomi Mitsuhashi; Norihiro Suzuki; Ichizo Nishino
Rheumatology (Oxford, England), 56(2), 287-293, Feb. 2017
Lead - Necklace cytoplasmic bodies in hereditary myopathy with early respiratory failure.
Akinori Uruha; Yukiko K Hayashi; Yasushi Oya; Madoka Mori-Yoshimura; Masahiro Kanai; Miho Murata; Mayumi Kawamura; Katsuhisa Ogata; Tsuyoshi Matsumura; Shigeaki Suzuki; Yukako Takahashi; Takayuki Kondo; Takeshi Kawarabayashi; Yuko Ishii; Norito Kokubun; Satoshi Yokoi; Rei Yasuda; Jun-ichi Kira; Satomi Mitsuhashi; Satoru Noguchi; Ikuya Nonaka; Ichizo Nishino
Journal of neurology, neurosurgery, and psychiatry, 86(5), 483-9, May 2015
Lead - Rituximab ameliorates anti-N-methyl-D-aspartate receptor encephalitis by removal of short-lived plasmablasts.
Yasuo Hachiya; Akinori Uruha; Emi Kasai-Yoshida; Konomi Shimoda; Ikuko Satoh-Shirai; Satoko Kumada; Eiji Kurihara; Kotoko Suzuki; Atsuko Ohba; Shin-ichiro Hamano; Hiroshi Sakuma
Journal of neuroimmunology, 265(1-2), 128-30, Dec. 2013
MISC
Books and other publications
- ここが知りたい!脳神経内科診療ハンドブック
井口, 正寛; 中森, 正博, Contributor, ミオパチー
中外医学社, May 2025
ISBN:9784498428324
https://webview.isho.jp/book/detail/abs/10.18886/9784498428324筋学を築き上げた人々
Fardeau, Michel; 岩田, 誠; 武田, 伸一, Joint editor
診断と治療社, Dec. 2021
ISBN:9784787825353脳神経内科医のための末梢神経・筋疾患診断トレーニング : 「電気生理×病理×画像」を読み解く30ケース
楠, 進; 園生, 雅弘; 清水, 潤, Contributor, 筋疾患 Case 2. 皮膚筋炎
南江堂, Jun. 2019
ISBN:9784524248155
https://webview.isho.jp/book/detail/abs/10.15106/9784524248155Research Themes
- Database establishment of small fiber neuropathy
Grants-in-Aid for Scientific Research, Japan Society for the Promotion of Science, Grant-in-Aid for Scientific Research (C)
TOKYO METROPOLITAN NEUROLOGICAL HOSPITAL
01 Apr. 2023 - 31 Mar. 2027 - Pathophysiology of sporadic late-onset nemaline myopathy - basis for therapeutic intervention
German Society for Muscle Diseases
2020 - 2022 - Search for autoantibodies in sporadic late-onset nemaline myopathy
Grant in aid for Survey Research, Japan Foundation for Neuroscience and Mental Health
Oct. 2016 - Sep. 2017 - Inflammasomes in the pathogenesis of inclusion body myositis
Grants-in-Aid for Scientific Research, Japan Society for the Promotion of Science, Grant-in-Aid for Young Scientists (B)
National Center of Neurology and Psychiatry
01 Apr. 2014 - 31 Mar. 2016 - Elucidation of the mechanism for formation of protein aggregates in skeletal myofibers with distal myopathy with rimmed vacuoles
Grants-in-Aid for Scientific Research, Japan Society for the Promotion of Science, Grant-in-Aid for Scientific Research (B)
National Center of Neurology and Psychiatry
01 Apr. 2011 - 31 Mar. 2015