関節拘縮が筋炎の顕在化に先行した若年成人発症の抗nuclear matrix protein 2（NXP-2）抗体陽性皮膚筋炎の1例
症例は23歳男性．18歳より両手の手指の関節拘縮に気づき，22歳より筋力低下と筋萎縮が出現した．皮疹，関節拘縮，嚥下障害，四肢体幹の筋力低下と著明な筋萎縮を認めた．間質性肺炎や悪性腫瘍はなく，腱のCT値の上昇を認めた．筋生検では筋束辺縁部萎縮を認めたが，リンパ球の浸潤はめだたなかった．血清抗体検査から，抗nuclear matrix protein 2（NXP-2）抗体陽性皮膚筋炎と診断した．ステロイド治療で，皮疹や球麻痺症状は速やかに消失したが，筋力低下や関節拘縮は残存した．若年成人発症で関節拘縮が4年間も先行した抗NXP-2抗体陽性皮膚筋炎の報告はなく，鑑別上重要である．...
Saved in:
| Published in | 臨床神経学 Vol. 64; no. 6; pp. 417 - 421 |
|---|---|
| Main Authors | , , , , , , , |
| Format | Journal Article |
| Language | Japanese |
| Published |
日本神経学会
2024
|
| Subjects | |
| Online Access | Get full text |
| ISSN | 0009-918X 1882-0654 |
| DOI | 10.5692/clinicalneurol.cn-001970 |
Cover
| Abstract | 症例は23歳男性．18歳より両手の手指の関節拘縮に気づき，22歳より筋力低下と筋萎縮が出現した．皮疹，関節拘縮，嚥下障害，四肢体幹の筋力低下と著明な筋萎縮を認めた．間質性肺炎や悪性腫瘍はなく，腱のCT値の上昇を認めた．筋生検では筋束辺縁部萎縮を認めたが，リンパ球の浸潤はめだたなかった．血清抗体検査から，抗nuclear matrix protein 2（NXP-2）抗体陽性皮膚筋炎と診断した．ステロイド治療で，皮疹や球麻痺症状は速やかに消失したが，筋力低下や関節拘縮は残存した．若年成人発症で関節拘縮が4年間も先行した抗NXP-2抗体陽性皮膚筋炎の報告はなく，鑑別上重要である． |
|---|---|
| AbstractList | 症例は23歳男性．18歳より両手の手指の関節拘縮に気づき，22歳より筋力低下と筋萎縮が出現した．皮疹，関節拘縮，嚥下障害，四肢体幹の筋力低下と著明な筋萎縮を認めた．間質性肺炎や悪性腫瘍はなく，腱のCT値の上昇を認めた．筋生検では筋束辺縁部萎縮を認めたが，リンパ球の浸潤はめだたなかった．血清抗体検査から，抗nuclear matrix protein 2（NXP-2）抗体陽性皮膚筋炎と診断した．ステロイド治療で，皮疹や球麻痺症状は速やかに消失したが，筋力低下や関節拘縮は残存した．若年成人発症で関節拘縮が4年間も先行した抗NXP-2抗体陽性皮膚筋炎の報告はなく，鑑別上重要である． |
| Author | 三浦, 叡人 小宅, 睦郎 種田, 朝音 藤田, 信也 西野, 一三 松下, 貴史 梅田, 能生 梅田, 麻衣子 |
| Author_xml | – sequence: 1 fullname: 小宅, 睦郎 organization: 長岡赤十字病院神経内科 – sequence: 1 fullname: 松下, 貴史 organization: 金沢大学皮膚科 – sequence: 1 fullname: 西野, 一三 organization: 国立精神・神経医療研究センター神経研究所疾病研究第一部 – sequence: 1 fullname: 梅田, 能生 organization: 長岡赤十字病院神経内科 – sequence: 1 fullname: 種田, 朝音 organization: 長岡赤十字病院神経内科 – sequence: 1 fullname: 梅田, 麻衣子 organization: 長岡赤十字病院神経内科 – sequence: 1 fullname: 三浦, 叡人 organization: 長岡赤十字病院神経内科 – sequence: 1 fullname: 藤田, 信也 organization: 長岡赤十字病院神経内科 |
| BookMark | eNpVkN9KAkEYxYcwyMx38AXWZmZn3d3LkP6BWBcF3i3jOFsr6yqrQt25QWUqEUSaEoRBFER200VJ9jKrrl71CilG0c13-ODHOZyzCHxW1uIAhBAMSxEVLzPTsAxGTYsX7awZZpYAIVJlOAf8SFGwACMS8QE_hFAVVKQkFkAwnzeS019SFUT8oDKu33kvpWH12nvruE7Ne656R-eu0xm3rwY3j4Na3XWeBsflUbvmOg3XuR1V7wfvr8PyRb_b9Zpdr3E6gYeVhlVkJqd2KEMLtnEQytnZAjesEP76KMcT28JEzyZUv3c5bvaGpQev1RmdtH7TUP-zugTmdWrmefBHA2B3bXUnuiHEttY3oysxIY1UQgUiykTmCtKTjGEsY51jzKiopGRKkE4YhjjFuIR1nSOGWYojKspMkoiKlZTIdTEA4jPfdL5A97iWs40MtQ81aheMSQft_6hahGiR6WGWNhv3D9yntpam4jf55aMA |
| ContentType | Journal Article |
| Copyright | 2024 日本神経学会 |
| Copyright_xml | – notice: 2024 日本神経学会 |
| DOI | 10.5692/clinicalneurol.cn-001970 |
| DatabaseTitleList | |
| DeliveryMethod | fulltext_linktorsrc |
| Discipline | Medicine |
| EISSN | 1882-0654 |
| EndPage | 421 |
| ExternalDocumentID | article_clinicalneurol_64_6_64_cn_001970_article_char_ja |
| GroupedDBID | ALMA_UNASSIGNED_HOLDINGS JSF OK1 P2P RJT |
| ID | FETCH-LOGICAL-j194a-43747e81fbcc2272fe22ca38d7a41f4c202dce52ffe1c2cde1a37c554928d3ef3 |
| ISSN | 0009-918X |
| IngestDate | Wed Sep 03 06:30:47 EDT 2025 |
| IsDoiOpenAccess | true |
| IsOpenAccess | true |
| IsPeerReviewed | true |
| IsScholarly | true |
| Issue | 6 |
| Language | Japanese |
| License | https://creativecommons.org/licenses/by-nc-nd/4.0/deed.ja |
| LinkModel | OpenURL |
| MergedId | FETCHMERGED-LOGICAL-j194a-43747e81fbcc2272fe22ca38d7a41f4c202dce52ffe1c2cde1a37c554928d3ef3 |
| OpenAccessLink | https://www.jstage.jst.go.jp/article/clinicalneurol/64/6/64_cn-001970/_article/-char/ja |
| PageCount | 5 |
| ParticipantIDs | jstage_primary_article_clinicalneurol_64_6_64_cn_001970_article_char_ja |
| PublicationCentury | 2000 |
| PublicationDate | 2024 |
| PublicationDateYYYYMMDD | 2024-01-01 |
| PublicationDate_xml | – year: 2024 text: 2024 |
| PublicationDecade | 2020 |
| PublicationTitle | 臨床神経学 |
| PublicationTitleAlternate | 臨床神経学 |
| PublicationYear | 2024 |
| Publisher | 日本神経学会 |
| Publisher_xml | – name: 日本神経学会 |
| References | 10) Blane CE, White SJ, Braunstein EM, et al. Patterns of calcification in childhood dermatomyositis. Am J Roentgenol 1984;142:397-400. 9) Tansley SL, Betteridge ZE, Shaddick G, et al. Calcinosis in juvenile dermatomyositis is influenced by both anti-NXP2 autoantibody status and age at disease onset. Rheumatology (Oxford) 2014;53:2204-2208. 12) Tanboon J, Inoue M, Saito Y, et al. Dermatomyositis: muscle pathology according to antibody subtypes. Neurology 2022;98:e739-e749. 4) Oddis CV, Fertig N, Goel A, et al. Clinical and serological characterization of the anti-MJ antibody in childhood myositis (abstr). Arthritis Rheum 1997;40:S139. 11) Albayda J, Pinal-Fernandez I, Huang W, et al. Antinuclear matrix protein 2 autoantibodies and edema, muscle disease, and malignancy risk in dermatomyositis patients. Arthritis Care Res (Hoboken) 2017;69:1771-1776. 1) Rider LG, Shah M, Mamyrova G, et al. The myositis autoantibody phenotypes of the juvenile idiopathic inflammatory myopathies. Medicine (Baltimore) 2013;92:223-243. 8) Guven M, Gholve PA, Blyakher A, et al. Juvenile dermatomyositis with bilateral progressive knee flexion contracture. Clin Orthop Relat Res 2007;464:238-241. 2) Rogers A, Chung L, Li S, et al. Cutaneous and systemic findings associated with nuclear matrix protein 2 antibodies in adult dermatomyositis patients. Arthritis Care Res (Hoboken) 2017;69:1909-1914. 7) Espada G, Maldonado Cocco JA, Fertig N, et al. Clinical and serologic characterization of an Argentine pediatric myositis cohort: identification of a novel autoantibody (anti-MJ) to a 142- kDa protein. J Rheumatol 2009;36:2547-2551. 6) Calado D, Moeda F, Hatia M, et al. Rehabilitation and exercise in dermatomyositis: a case report and narrative review of the literature. Cureus 2022;14:e33034. 3) Ichimura Y, Konishi R, Shobo M, et al. Anti-nuclear matrix protein 2 antibody-positive inflammatory myopathies represent extensive myositis without dermatomyositis-specific rash. Rheumatology (Oxford) 2022;61:1222-1227. 5) 埜中征哉，西野一三．臨床のための筋病理 第5版．東京：日本医学新報社；2021. p. 224. |
| References_xml | – reference: 2) Rogers A, Chung L, Li S, et al. Cutaneous and systemic findings associated with nuclear matrix protein 2 antibodies in adult dermatomyositis patients. Arthritis Care Res (Hoboken) 2017;69:1909-1914. – reference: 3) Ichimura Y, Konishi R, Shobo M, et al. Anti-nuclear matrix protein 2 antibody-positive inflammatory myopathies represent extensive myositis without dermatomyositis-specific rash. Rheumatology (Oxford) 2022;61:1222-1227. – reference: 4) Oddis CV, Fertig N, Goel A, et al. Clinical and serological characterization of the anti-MJ antibody in childhood myositis (abstr). Arthritis Rheum 1997;40:S139. – reference: 6) Calado D, Moeda F, Hatia M, et al. Rehabilitation and exercise in dermatomyositis: a case report and narrative review of the literature. Cureus 2022;14:e33034. – reference: 1) Rider LG, Shah M, Mamyrova G, et al. The myositis autoantibody phenotypes of the juvenile idiopathic inflammatory myopathies. Medicine (Baltimore) 2013;92:223-243. – reference: 7) Espada G, Maldonado Cocco JA, Fertig N, et al. Clinical and serologic characterization of an Argentine pediatric myositis cohort: identification of a novel autoantibody (anti-MJ) to a 142- kDa protein. J Rheumatol 2009;36:2547-2551. – reference: 8) Guven M, Gholve PA, Blyakher A, et al. Juvenile dermatomyositis with bilateral progressive knee flexion contracture. Clin Orthop Relat Res 2007;464:238-241. – reference: 12) Tanboon J, Inoue M, Saito Y, et al. Dermatomyositis: muscle pathology according to antibody subtypes. Neurology 2022;98:e739-e749. – reference: 10) Blane CE, White SJ, Braunstein EM, et al. Patterns of calcification in childhood dermatomyositis. Am J Roentgenol 1984;142:397-400. – reference: 11) Albayda J, Pinal-Fernandez I, Huang W, et al. Antinuclear matrix protein 2 autoantibodies and edema, muscle disease, and malignancy risk in dermatomyositis patients. Arthritis Care Res (Hoboken) 2017;69:1771-1776. – reference: 9) Tansley SL, Betteridge ZE, Shaddick G, et al. Calcinosis in juvenile dermatomyositis is influenced by both anti-NXP2 autoantibody status and age at disease onset. Rheumatology (Oxford) 2014;53:2204-2208. – reference: 5) 埜中征哉，西野一三．臨床のための筋病理 第5版．東京：日本医学新報社；2021. p. 224. |
| SSID | ssib000959814 ssib058494234 ssib002821941 ssib000940416 ssib002484599 ssib005879791 ssj0060813 |
| Score | 2.4137802 |
| Snippet | 症例は23歳男性．18歳より両手の手指の関節拘縮に気づき，22歳より筋力低下と筋萎縮が出現した．皮疹，関節拘縮，嚥下障害，四肢体幹の筋力低下と著明な筋萎縮を認めた．... |
| SourceID | jstage |
| SourceType | Publisher |
| StartPage | 417 |
| SubjectTerms | 抗nuclear matrix protein 2（NXP-2）抗体 皮膚筋炎 若年成人発症 関節拘縮 |
| Title | 関節拘縮が筋炎の顕在化に先行した若年成人発症の抗nuclear matrix protein 2（NXP-2）抗体陽性皮膚筋炎の1例 |
| URI | https://www.jstage.jst.go.jp/article/clinicalneurol/64/6/64_cn-001970/_article/-char/ja |
| Volume | 64 |
| hasFullText | 1 |
| inHoldings | 1 |
| isFullTextHit | |
| isPrint | |
| ispartofPNX | 臨床神経学, 2024, Vol.64(6), pp.417-421 |
| journalDatabaseRights | – providerCode: PRVAFT databaseName: Open Access Digital Library customDbUrl: eissn: 1882-0654 dateEnd: 99991231 omitProxy: true ssIdentifier: ssib000959814 issn: 0009-918X databaseCode: KQ8 dateStart: 20080101 isFulltext: true titleUrlDefault: http://grweb.coalliance.org/oadl/oadl.html providerName: Colorado Alliance of Research Libraries |
| link | http://utb.summon.serialssolutions.com/2.0.0/link/0/eLvHCXMwrR1da9RAMJQK4ov4id_cg_tUUi-bTXb3MbnmLEKLSgt9C7l8oIeeUloQn3qCWntFBLG1RZAKoiDWFx-0tP6Z6921T_4FZza5a1qsaBFCmOzMzs7O7mZnlt1ZTbvMWRyFUZTokhWZzmQx1AOe4Lgq8rAiwYKIcB1yZNQeHmfXJqyJvv6buV1L01OVwfDhb8-VHKRVIQ3aFU_J_kPL9phCAsDQvvCGFob3X7Ux8SSRNnEo8ThxyrhrwbOJcIkUmOIK4njEM8FaJKKkaIYQC4CgRHRRSCOJYxBpEc8iskQcgQBkAeYZjatS4AGUQGJkqFCSd4EyooC_o_i4krhMySOIBMEYcR18oHQpuwBIwnNiALEDiTUMshxMDtzF-wMeDKhYErdrA1DNMnFLwHB04rre-5K9fKqQISJNpRiJMKKKxOGqNEcVAjLaCO-vD0Mx8gCXt91VTp4pB-QXKQvQmKe0rTQGKGDq9NY7FW2RiLLCeKhC6NRKGiRDQQWU6u3Q24iBwlEEpU5FL4hLlT4t5OXSHXqBIrhlxYljLRR9mrnYBWSeP_QXEANFYChbxh_EQH2pVFnOrwbRnXVgJZ6qNALQU0p_0IEqHDqRs2tqlDBC1T3PYBiksyG6X3j6OD9dpkHns99Cfu5j6SHczIxi6cH3vTO0ZUuM-Ns99quCtt4ZDGs6-hrpFTJ74p9no8vfncW3mW_jK6z5aVa_R3grmPSr4DMdohysT9yocSPncuBPKe8SSUuK3E4CygSz5K4lCSN_1tsSXPKd-FJgv0vwSFjX-rPBxO7d3ojqTHcPYrWv7FdpMH6r4Ap2t5Eqy3bsmHY0c0kLTlqx41pfNTihHR7JNt2c1Oa2F951vsy0G68731ab9fnO50bn0fNmfXV75VXrzcfW_EKz_qn1eHZrZb5ZX2zW32413re-f23PvthcW-ssrXUWnwJxe24xG9aFdFgXsmFdoD_XZ9WA_rn-DKg2N15uL220Zz50lle3niz3SjM2fzROaeNlb6w0rGdXuOhV0FqgM5MzHgsjqYQhpZwmMaVhYIqIB8xIWAhdOApjiyZJbIQ0jGIjMHloYdhIEZlxYp7W-mv3avEZrRBVWIXGJhUVxpjNYmmFeLkGeCgJcGLirHY11aJ_P43T4x-055z7b5zOa0dwkKZLthe0_qnJ6fgiODFTlUuqV_4CYGcgqQ |
| linkProvider | Colorado Alliance of Research Libraries |
| openUrl | ctx_ver=Z39.88-2004&ctx_enc=info%3Aofi%2Fenc%3AUTF-8&rfr_id=info%3Asid%2Fsummon.serialssolutions.com&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.atitle=%E9%96%A2%E7%AF%80%E6%8B%98%E7%B8%AE%E3%81%8C%E7%AD%8B%E7%82%8E%E3%81%AE%E9%A1%95%E5%9C%A8%E5%8C%96%E3%81%AB%E5%85%88%E8%A1%8C%E3%81%97%E3%81%9F%E8%8B%A5%E5%B9%B4%E6%88%90%E4%BA%BA%E7%99%BA%E7%97%87%E3%81%AE%E6%8A%97nuclear+matrix+protein+2%EF%BC%88NXP-2%EF%BC%89%E6%8A%97%E4%BD%93%E9%99%BD%E6%80%A7%E7%9A%AE%E8%86%9A%E7%AD%8B%E7%82%8E%E3%81%AE1%E4%BE%8B&rft.jtitle=%E8%87%A8%E5%BA%8A%E7%A5%9E%E7%B5%8C%E5%AD%A6&rft.au=%E5%B0%8F%E5%AE%85%2C+%E7%9D%A6%E9%83%8E&rft.au=%E6%9D%BE%E4%B8%8B%2C+%E8%B2%B4%E5%8F%B2&rft.au=%E8%A5%BF%E9%87%8E%2C+%E4%B8%80%E4%B8%89&rft.au=%E6%A2%85%E7%94%B0%2C+%E8%83%BD%E7%94%9F&rft.date=2024&rft.pub=%E6%97%A5%E6%9C%AC%E7%A5%9E%E7%B5%8C%E5%AD%A6%E4%BC%9A&rft.issn=0009-918X&rft.eissn=1882-0654&rft.volume=64&rft.issue=6&rft.spage=417&rft.epage=421&rft_id=info:doi/10.5692%2Fclinicalneurol.cn-001970&rft.externalDocID=article_clinicalneurol_64_6_64_cn_001970_article_char_ja |
| thumbnail_l | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/lc.gif&issn=0009-918X&client=summon |
| thumbnail_m | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/mc.gif&issn=0009-918X&client=summon |
| thumbnail_s | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/sc.gif&issn=0009-918X&client=summon |