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LIBRIS Formathandbok  (Information om MARC21)
FältnamnIndikatorerMetadata
00003975naa a2200697 4500
001oai:prod.swepub.kib.ki.se:150871366
003SwePub
008240913s2023 | |||||||||||000 ||eng|
024a http://kipublications.ki.se/Default.aspx?queryparsed=id:1508713662 URI
024a https://doi.org/10.1136/ard-2022-2229352 DOI
040 a (SwePub)ki
041 a engb eng
042 9 SwePub
072 7a ref2 swepub-contenttype
072 7a art2 swepub-publicationtype
100a Zhou, D4 aut
2451 0a Low copy numbers of complement C4 and C4A deficiency are risk factors for myositis, its subgroups and autoantibodies
264 c 2022-09-28
264 1b BMJ,c 2023
520 a Idiopathic inflammatory myopathies (IIM) are a group of autoimmune diseases characterised by myositis-related autoantibodies plus infiltration of leucocytes into muscles and/or the skin, leading to the destruction of blood vessels and muscle fibres, chronic weakness and fatigue. While complement-mediated destruction of capillary endothelia is implicated in paediatric and adult dermatomyositis, the complex diversity of complementC4in IIM pathology was unknown.MethodsWe elucidated the gene copy number (GCN) variations of totalC4,C4AandC4B, longandshort genesin 1644 Caucasian patients with IIM, plus 3526 matched healthy controls using real-time PCR or Southern blot analyses. Plasma complement levels were determined by single radial immunodiffusion.ResultsThe large study populations helped establish the distribution patterns of variousC4GCN groups. Low GCNs ofC4T(C4T=2+3) andC4Adeficiency (C4A=0+1) were strongly correlated with increased risk of IIM with OR equalled to 2.58 (2.28–2.91), p=5.0×10−53forC4T, and 2.82 (2.48–3.21), p=7.0×10−57forC4Adeficiency. Contingency and regression analyses showed that among patients withC4Adeficiency, the presence ofHLA-DR3became insignificant as a risk factor in IIM except for inclusion body myositis (IBM), by which 98.2% hadHLA-DR3with an OR of 11.02 (1.44–84.4). Intragroup analyses of patients with IIM for C4 protein levels and IIM-related autoantibodies showed that those with anti-Jo-1 or with anti-PM/Scl had significantly lower C4 plasma concentrations than those without these autoantibodies.ConclusionsC4Adeficiency is relevant in dermatomyositis,HLA-DRB1*03is important in IBM and bothC4Adeficiency andHLA-DRB1*03contribute interactively to risk of polymyositis.
700a King, EH4 aut
700a Rothwell, S4 aut
700a Krystufkova, O4 aut
700a Notarnicola, Au Karolinska Institutet4 aut
700a Coss, S4 aut
700a Abdul-Aziz, R4 aut
700a Miller, KE4 aut
700a Dang, A4 aut
700a Yu, GR4 aut
700a Drew, J4 aut
700a Lundstrom, E4 aut
700a Pachman, LM4 aut
700a Mamyrova, G4 aut
700a Curiel, R4 aut
700a De Paepe, B4 aut
700a De Bleecker, JL4 aut
700a Payton, A4 aut
700a Ollier, W4 aut
700a O'Hanlon, TP4 aut
700a Targoff, IN4 aut
700a Flegel, WA4 aut
700a Sivaraman, V4 aut
700a Oberle, E4 aut
700a Akoghlanian, S4 aut
700a Driest, K4 aut
700a Spencer, CH4 aut
700a Wu, YL4 aut
700a Nagaraja, HN4 aut
700a Ardoin, SP4 aut
700a Chinoy, H4 aut
700a Rider, LG4 aut
700a Miller, FW4 aut
700a Lundberg, IEu Karolinska Institutet4 aut
700a Padyukov, Lu Karolinska Institutet4 aut
700a Vencovsky, J4 aut
700a Lamb, JA4 aut
700a Yu, CY4 aut
710a Karolinska Institutet4 org
773t Annals of the rheumatic diseasesd : BMJg 82:2, s. 235-245q 82:2<235-245x 1468-2060x 0003-4967
8564 8u http://kipublications.ki.se/Default.aspx?queryparsed=id:150871366
8564 8u https://doi.org/10.1136/ard-2022-222935

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