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High maternal expression of SIGLEC1 on monocytes as a surrogate marker of a type I interferon signature is a risk factor for the development of autoimmune congenital heart block
  1. Anna R Lisney1,2,
  2. Franziska Szelinski1,
  3. Karin Reiter1,
  4. Gerd R Burmester1,
  5. Thomas Rose1,2,
  6. Thomas Dörner1,2
  1. 1 Department of Rheumatology and Clinical Immunology, Charité Universitätsmedizin Berlin, Berlin, Germany
  2. 2 German Rheumatism Research Center Berlin (DRFZ), Berlin, Germany
  1. Correspondence to Professor Thomas Dörner, Department of Rheumatology and Clinical Immunology, Charité Universitätsmedizin Berlin, Charitéplatz 1, 10117 Berlin, Germany; thomas.doerner{at}


Objectives Autoimmune congenital heart block (CHB) is associated with placental transcytosis of maternal autoantibodies directed against Ro/SS-A and La/SS-B. However, only about 2% of children born to mothers with the respective antibodies are affected, indicating that further risk factors exist, which are not yet fully understood. In this study, we investigated whether a maternal type I interferon (IFN) signature represents a risk factor for the development of CHB.

Methods Blood samples, clinical data and serological parameters from 9 women with CHB pregnancies, 14 pregnant women with antibodies against Ro/SS-A but without a CHB complication and another 30 healthy pregnant women as controls were studied. SIGLEC1 expression was measured by flow cytometry and was correlated to plasma IFN-α levels measured by ELISA, and IFN-γ-induced protein 10 (IP-10) levels measured by Bio-Plex technique.

Results Mothers of affected children had a significantly higher expression of SIGLEC1 (p=0.0034) and IFN-α (p=0.014), but not of IP-10 (p=0.14, all MWU) compared to mothers of unaffected children. SIGLEC1 and IFN-α expression were reduced by hydroxychloroquine and oral glucocorticoids.

Conclusions High expression of SIGLEC1 in pregnant women with autoantibodies against Ro/SS-A indicates an enhanced risk for CHB development, and these women may benefit especially from IFN-α directed therapy, for example with hydroxychloroquine.

  • congenital heart block
  • neonatal lupus syndrome
  • sialoadhesin
  • interferon-α

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  • Contributors ARL, FS, KR, TR and TD: conception, design, analysis and interpretation of data.

    ARL: drafting the article.

    TD, TR and GRB: revising the manuscript critically for important intellectual content.

    All authors read and approved the final manuscript.

  • Funding CRC initiative of the German Research Foundation (DFG) (SFB 650; TD and GRB). ARL receives a scholarship from the Leibniz Graduate School for Rheumatology (DRFZ).

  • Competing interests None declared.

  • Patient consent An own consent form approved by the ethics committee of the Charite Universitätsmedizin Berlin was used and signed by each study participant.

  • Ethics approval Ethics committee of the Charite Universitätsmedizin Berlin.

  • Provenance and peer review Not commissioned; externally peer reviewed.