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Epidemiology of Primary Immunodeficiency in Iceland

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Abstract

Purpose

Primary immunodeficiencies (PID) are rare heterogeneous diseases. Little is known about the prevalence of PID in Iceland and no national registry exists. The aim of the study was to describe the epidemiology of PID in Iceland.

Methods

Using The European Society’s for Immunodeficiencies (ESID) criteria for PID, information about individuals with a known PID between 1990 and 2010 in Iceland were collected from inpatient registries of the National University Hospital of Iceland, the Department of Immunology and from clinical immunologists. Selective IgA deficiency, mannan binding lectin deficiency and secondary immunodeficiencies were excluded

Results

Sixty six individuals met the study criteria, 35 of them (53 %) were females. Four patients died during the study period from PID- or treatment related complications and two moved abroad. In the beginning of 2011 there were 60 individuals living in Iceland with a known PID diagnosis meeting ESID’s criteria. Estimated prevalence for PID in the Icelandic population of 318.452 habitants was 18.8 for 100.000 inhabitants. Predominantly antibody disorders comprised the largest category of PID in Iceland.

Conclusions

The prevalence of PID is high in Iceland compared to reports from other nations. Our patient data are easily accessible and a central laboratory measures the immune parameters. This high prevalence may indicate that PID is more common than generally recognized.

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Conflicts of Interest

None of the authors has any conflicts of interest to report regarding this work and no funding was received for this study. Three authors (BRL, STS and AH) have received research grants and travel grants from pharmaceutical companies, not related to this study.

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Authors and Affiliations

  1. Department of Immunology, Landspitali—The National University Hospital of Iceland, Hringbraut (building 14 at Eiriksgata), 101, Reykjavik, Iceland

    Bjorn R. Ludviksson, Sigurveig T. Sigurdardottir & Thorgeir O. Hardarson

  2. Department of Genetics and Molecular Medicine, Landspitali - The National University Hospital of Iceland, Hringbraut (building 7 at Baronstigur), 101, Reykjavik, Iceland

    Johann Heidar Johannsson

  3. Children’s Hospital Iceland, Landspitali—University Hospital, 101, Reykjavík, Iceland

    Asgeir Haraldsson

Authors
  1. Bjorn R. Ludviksson
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  2. Sigurveig T. Sigurdardottir
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  3. Johann Heidar Johannsson
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  4. Asgeir Haraldsson
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  5. Thorgeir O. Hardarson
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Corresponding author

Correspondence to Bjorn R. Ludviksson.

Additional information

All authors have seen and approved the manuscript and contributed significantly to the work. The manuscript has not been previously published nor is it being considered for publication elsewhere.

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Ludviksson, B.R., Sigurdardottir, S.T., Johannsson, J.H. et al. Epidemiology of Primary Immunodeficiency in Iceland. J Clin Immunol 35, 75–79 (2015). https://doi.org/10.1007/s10875-014-0107-3

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  • DOI: https://doi.org/10.1007/s10875-014-0107-3

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