{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,28]],"date-time":"2025-10-28T10:28:37Z","timestamp":1761647317566},"reference-count":16,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2004,10,8]],"date-time":"2004-10-08T00:00:00Z","timestamp":1097193600000},"content-version":"vor","delay-in-days":3417,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Annals of Neurology"],"published-print":{"date-parts":[[1995,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Inclusion body myositis developed in two men, 36 and 48 years old with long\u2010standing common variable immunodeficiency. Immunophenotypic analysis of the endomysial cells showed an increased number of natural killer (NK) cells (defined as CD57<jats:sup>+<\/jats:sup>, CD56<jats:sup>+<\/jats:sup>, CD3<jats:sup>\u2212<\/jats:sup>, CD8<jats:sup>\u2212<\/jats:sup>, CD68<jats:sup>\u2212<\/jats:sup>) accounting for 8.5 to 9.5% of the total cells, compared with a mean of 1% in sporadic inclusion body myositis. The remaining cells were CD8<jats:sup>+<\/jats:sup>, macrophages, and CD4<jats:sup>+<\/jats:sup> T cells. NK cells were positive for intercellular cell adhesion molecule\u20101 and invaded muscle fibers negative for major histocompatibility complex (MHC) class I. In contrast to ubiquitous endomysial expression of MHC class I antigen in sporadic inclusion body myositis, the MHC class I in common variable immunodeficiency and inclusion body myositis was absent or weakly expressed in only some of the muscle fibers surrounded by CD8<jats:sup>+<\/jats:sup> cells. Enteroviral or retroviral RNA sequences were not amplified. Treatment with intravenous immunoglobulin improved strength in 1 patient whose repeated muscle biopsy specimen showed normal NK cells. We conclude that inclusion body myositis can develop in patients with common variable immunodeficiency. Common variable immunodeficiency with inclusion body myositis is an immune myopathy mediated by NK cells in a non\u2013MHC class I\u2013restricted cytotoxicity, and by CD8<jats:sup>+<\/jats:sup> cells in an MHC class I\u2013restricted process. This is the first description of an inflammatory myopathy in which NK cells participate in the myocytotoxic process.<\/jats:p>","DOI":"10.1002\/ana.410370615","type":"journal-article","created":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T13:58:48Z","timestamp":1104587928000},"page":"806-810","source":"Crossref","is-referenced-by-count":34,"title":["Common variable immunodeficiency and inclusion body myositis: A distinct myopathy mediated by natural killer cells"],"prefix":"10.1002","volume":"37","author":[{"given":"Marinos C.","family":"Dalakas","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Isabel","family":"Illa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2004,10,8]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199111213252107"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1055\/s-2008-1041132"},{"key":"e_1_2_1_4_2","first-page":"1384","volume-title":"Myology: basic and clinical","author":"Mikol J.","year":"1994"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410160207"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-118-9-199305010-00011"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410360414"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410290505"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410310109"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199312303292704"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.43.5.876"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1084\/jem.173.6.1451"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410230111"},{"key":"e_1_2_1_14_2","doi-asserted-by":"crossref","first-page":"2140","DOI":"10.4049\/jimmunol.142.6.2140","article-title":"Susceptibility to natural killer cell\u2010mediated cytolysis is independent of the level of target cell class I HLA expression","volume":"142","author":"Leiden JM","year":"1989","journal-title":"J Immunol"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199103283241303"},{"key":"e_1_2_1_16_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI114711"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2249.1992.tb05826.x"}],"container-title":["Annals of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fana.410370615","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fana.410370615","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ana.410370615","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,26]],"date-time":"2023-10-26T23:10:18Z","timestamp":1698361818000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ana.410370615"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1995,6]]},"references-count":16,"journal-issue":{"issue":"6","published-print":{"date-parts":[[1995,6]]}},"alternative-id":["10.1002\/ana.410370615"],"URL":"https:\/\/doi.org\/10.1002\/ana.410370615","archive":["Portico"],"relation":{},"ISSN":["0364-5134","1531-8249"],"issn-type":[{"value":"0364-5134","type":"print"},{"value":"1531-8249","type":"electronic"}],"subject":[],"published":{"date-parts":[[1995,6]]}}}