Quality of Life Research

, Volume 23, Issue 2, pp 645–658 | Cite as

Health-related quality of life (HRQL) in immunodeficient adults with selective IgA deficiency compared with age- and gender-matched controls and identification of risk factors for poor HRQL

  • G. H. Jörgensen
  • A. Gardulf
  • M. I. Sigurdsson
  • S. Arnlaugsson
  • L. Hammarström
  • B. R. Ludviksson



Selective IgA deficiency (SIgAD) is the most common primary immunodeficiency with a prevalence of 1/600 in the general population. Any targeted health-related quality of life (HRQL) study of adults with SIgAD has never been presented. The objectives of the study were to compare HRQL between SIgAD adults and randomly selected age- and gender-matched population controls, and to identify risk factors for poor HRQL.


Thirty-two SIgAD individuals and 63 controls answered three questionnaires (clinical data, Short Form-36 Health Survey (SF-36), infection-related HRQL) at baseline before undergoing medical/dental examinations and laboratory assessments. HRQL in SIgAD was re-evaluated after 6 and 12 months.


Baseline: Selective IgA deficiency individuals reported significantly increased fear of contracting infections (p < 0.01). Those scoring high on fear also perceived significantly poorer physical health (p < 0.01). SF-36 results indicated that SIgAD individuals perceived poorer HRQL, although this was not statistically significant. Follow-up: Compared with SF-36 responses at baseline, SIgAD individuals reported significantly more pain (p < 0.01) at 6 months, poorer general health (p < 0.05) and summarised physical HRQL (p < 0.01) at 6 and 12 months and decreased vitality at 12 months. The summarised mental scale remained stable over time. Risk factors for poor HRQL: The number of antibiotic treatments during the previous year (p < 0.001), number of daily medications (p < 0.01), allergic rhinoconjunctivitis (p < 0.05), chronic musculoskeletal symptoms at least every week (p < 0.05) and anxiety and/or insomnia (p < 0.05) were identified as independent risk factors for poor HRQL.


The study highlights the importance of identifying and thoroughly evaluating, educating and following up individuals with SIgAD, as their HRQL may be negatively affected due to health problems possible to prevent and treat.


Primary immunodeficiency IgA deficiency Quality of life SF-36 Patient-reported outcomes Risk factors 



Selective IgA deficiency


Primary immunodeficiency


Health-related quality of life


Short Form-36 Health Survey


Common variable immunodeficiency


X-linked agammaglobulinaemia


Physical functioning




Bodily pain


General health


Physical component summary score




Social functioning




Mental health


Mental component summary score




Body mass index



We are grateful to all the individuals participating in the study, and to Hilary Hocking, Östersund, Sweden, for language revision. This study was supported by the Landspitali University Hospital Research Fund, the Icelandic Research Fund, Karolinska Institutet (KI Fonder), Stockholm, Sweden, and Division of Research and Development, Primary Care, County Council of Uppsala, Sweden.

Conflict of interest

No conflict of interest is reported for any of the authors.


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Copyright information

© Springer Science+Business Media Dordrecht 2013

Authors and Affiliations

  • G. H. Jörgensen
    • 1
    • 2
  • A. Gardulf
    • 3
  • M. I. Sigurdsson
    • 1
  • S. Arnlaugsson
    • 4
  • L. Hammarström
    • 3
  • B. R. Ludviksson
    • 1
    • 2
  1. 1.Department of MedicineUniversity of IcelandReykjavíkIceland
  2. 2.Department of ImmunologyLandspitali University HospitalReykjavíkIceland
  3. 3.Division of Clinical Immunology at the Department of Laboratory MedicineKarolinska Institutet at Karolinska University HospitalHuddinge, StockholmSweden
  4. 4.Faculty of OdontologyUniversity of IcelandReykjavíkIceland

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