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Low copy numbers of complement C4 and C4A deficiency are risk factors for myositis, its subgroups and autoantibodies

The result's identifiers

  • Result code in IS VaVaI

    <a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00023728%3A_____%2F23%3AN0000048" target="_blank" >RIV/00023728:_____/23:N0000048 - isvavai.cz</a>

  • Result on the web

    <a href="https://doi.org/10.1136/ard-2022-222935" target="_blank" >https://doi.org/10.1136/ard-2022-222935</a>

  • DOI - Digital Object Identifier

    <a href="http://dx.doi.org/10.1136/ard-2022-222935" target="_blank" >10.1136/ard-2022-222935</a>

Alternative languages

  • Result language

    angličtina

  • Original language name

    Low copy numbers of complement C4 and C4A deficiency are risk factors for myositis, its subgroups and autoantibodies

  • Original language description

    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 complement C4 in IIM pathology was unknown. Methods We elucidated the gene copy number (GCN) variations of total C4, C4A and C4B, long and short genes in 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. Results The large study populations helped establish the distribution patterns of various C4 GCN groups. Low GCNs of C4T (C4T=2+3) and C4A deficiency (C4A=0+1) were strongly correlated with increased risk of IIM with OR equalled to 2.58 (2.28-2.91), p=5.0x10(-53) for C4T, and 2.82 (2.48-3.21), p=7.0x10(-57) for C4A deficiency. Contingency and regression analyses showed that among patients with C4A deficiency, the presence of HLA-DR3 became insignificant as a risk factor in IIM except for inclusion body myositis (IBM), by which 98.2% had HLA-DR3 with 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. Conclusions C4A deficiency is relevant in dermatomyositis, HLA-DRB1*03 is important in IBM and both C4A deficiency and HLA-DRB1*03 contribute interactively to risk of polymyositis.

  • Czech name

  • Czech description

Classification

  • Type

    J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database

  • CEP classification

  • OECD FORD branch

    30226 - Rheumatology

Result continuities

  • Project

  • Continuities

    I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Others

  • Publication year

    2023

  • Confidentiality

    S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Data specific for result type

  • Name of the periodical

    Annals of the Rheumatic Diseases.

  • ISSN

    0003-4967

  • e-ISSN

    1468-2060

  • Volume of the periodical

    82

  • Issue of the periodical within the volume

    2

  • Country of publishing house

    GB - UNITED KINGDOM

  • Number of pages

    11

  • Pages from-to

    235-245

  • UT code for WoS article

    000863385900001

  • EID of the result in the Scopus database

    2-s2.0-85142036914