Chiseko Ikenaga1, Akatsuki Kubota1, Masato Kadoya1, Kenichiro Taira1, Naohiro Uchio1, Ayumi Hida1, Meiko Hashimoto Maeda1, Yu Nagashima1, Hiroyuki Ishiura1, Kenichi Kaida1, Jun Goto1, Shoji Tsuji1, Jun Shimizu2. 1. From the Department of Neurology (C.I., A.K., K.T., N.U., M.H.M., Y.N., H.I., S.T., J.S.), Graduate School of Medicine, the University of Tokyo; Division of Neurology (M.K., K.K.), Department of Internal Medicine, National Defense Medical College, Saitama; Department of Neurology (A.H.), National Center for Global Health and Medicine; Department of Neurology (M.H.M.), Federation of National Public Service Personnel Mutual Aid Associations Toranomon Hospital; Okinaka Memorial Institute For Medical Research (M.H.M.); and Department of Neurology (J.G.), International University of Health and Welfare, Mita Hospital, Tokyo, Japan. 2. From the Department of Neurology (C.I., A.K., K.T., N.U., M.H.M., Y.N., H.I., S.T., J.S.), Graduate School of Medicine, the University of Tokyo; Division of Neurology (M.K., K.K.), Department of Internal Medicine, National Defense Medical College, Saitama; Department of Neurology (A.H.), National Center for Global Health and Medicine; Department of Neurology (M.H.M.), Federation of National Public Service Personnel Mutual Aid Associations Toranomon Hospital; Okinaka Memorial Institute For Medical Research (M.H.M.); and Department of Neurology (J.G.), International University of Health and Welfare, Mita Hospital, Tokyo, Japan. jshimizu-tky@umin.ac.jp.
Abstract
OBJECTIVE: To determine the clinical features of myositis patients with the histopathologic finding of CD8-positive T cells invading non-necrotic muscle fibers expressing major histocompatibility complex class 1 (CD8-MHC-1 complex), which is shared by polymyositis (PM) and inclusion body myositis (IBM), in relation to the p62 immunostaining pattern of muscle fibers. METHODS: All 93 myositis patients with CD8-MHC-1 complex who were referred to our hospital from 1993 to 2015 were classified on the basis of the European Neuromuscular Center (ENMC) diagnostic criteria for IBM (Rose, 2013) or PM (Hoogendijk, 2004) and analyzed. RESULTS: The 93 patients included were 17 patients with PM, 70 patients with IBM, and 6 patients who neither met the criteria for PM nor IBM in terms of muscle weakness distribution (unclassifiable group). For these PM, IBM, and unclassifiable patients, their mean ages at diagnosis were 63, 70, and 64 years; autoimmune disease was present in 7 (41%), 13 (19%), and 4 (67%); hepatitis C virus infection was detected in 0%, 13 (20%), and 2 (33%); and p62 was immunopositive in 0%, 66 (94%), and 2 (33%), respectively. Of the treated patients, 11 of 16 PM patients and 4 of 6 p62-immunonegative patients in the unclassifiable group showed responses to immunotherapy, whereas all 44 patients with IBM and 2 p62-immunopositive patients in the unclassifiable group were unresponsive to immunotherapy. CONCLUSIONS: CD8-MHC-1 complex is present in patients with PM, IBM, or unclassifiable group. The data may serve as an argument for a trial of immunosuppressive treatment in p62-immunonegative patients with unclassifiable myositis.
OBJECTIVE: To determine the clinical features of myositispatients with the histopathologic finding of CD8-positive T cells invading non-necrotic muscle fibers expressing major histocompatibility complex class 1 (CD8-MHC-1 complex), which is shared by polymyositis (PM) and inclusion body myositis (IBM), in relation to the p62 immunostaining pattern of muscle fibers. METHODS: All 93 myositispatients with CD8-MHC-1 complex who were referred to our hospital from 1993 to 2015 were classified on the basis of the European Neuromuscular Center (ENMC) diagnostic criteria for IBM (Rose, 2013) or PM (Hoogendijk, 2004) and analyzed. RESULTS: The 93 patients included were 17 patients with PM, 70 patients with IBM, and 6 patients who neither met the criteria for PM nor IBM in terms of muscle weakness distribution (unclassifiable group). For these PM, IBM, and unclassifiable patients, their mean ages at diagnosis were 63, 70, and 64 years; autoimmune disease was present in 7 (41%), 13 (19%), and 4 (67%); hepatitis C virus infection was detected in 0%, 13 (20%), and 2 (33%); and p62 was immunopositive in 0%, 66 (94%), and 2 (33%), respectively. Of the treated patients, 11 of 16 PM patients and 4 of 6 p62-immunonegative patients in the unclassifiable group showed responses to immunotherapy, whereas all 44 patients with IBM and 2 p62-immunopositive patients in the unclassifiable group were unresponsive to immunotherapy. CONCLUSIONS:CD8-MHC-1 complex is present in patients with PM, IBM, or unclassifiable group. The data may serve as an argument for a trial of immunosuppressive treatment in p62-immunonegative patients with unclassifiable myositis.
Authors: Mridul Johari; Anna Vihola; Johanna Palmio; Manu Jokela; Per Harald Jonson; Jaakko Sarparanta; Sanna Huovinen; Marco Savarese; Peter Hackman; Bjarne Udd Journal: J Neurol Date: 2022-03-02 Impact factor: 6.682
Authors: Chiseko Ikenaga; Hidetoshi Date; Motoi Kanagawa; Jun Mitsui; Hiroyuki Ishiura; Jun Yoshimura; Iago Pinal-Fernandez; Andrew L Mammen; Thomas E Lloyd; Shoji Tsuji; Jun Shimizu; Tatsushi Toda; Jun Goto Journal: Ann Neurol Date: 2022-02-11 Impact factor: 11.274