Adults with selective IgA deficiency
- Health-related quality of life (HRQL)-Risk factors for poor HRQL
ESID-INGID-IPOPI Meeting 2012
Ann Gardulf
HRQL in selective IgA deficiency
Dept of Medicine, University of Iceland, Dept of Immunology, Landspitali-University Hospital, Reykjavík, Iceland
and Dept of Laboratory Medicine, Section of Clinical
Immunology, Karolinska Institutet, Huddinge, Stockholm, Sweden
Guðmundur Jorgensen Ann Gardulf
Sigurdsson M. I., Sigurðardóttir S., Thorsteinsdottir I., Gudmundsson S., Hammarström L. and Ludviksson B.R.
HRQL in selective IgA deficiency
Selective IgA deficiency (SIgAD) is the most common primary immunodeficiency
Definition: serum IgA <0.07 g/L
Estimated prevalence: 1/600
HRQL in selective IgA deficiency
Selective IgA deficiency (SIgAD) is the most common primary immunodeficiency
Definition: serum IgA <0.07 g/L
Estimated prevalence: 1/600
Nordic countries 41,000 individualsUSA 511,000 individualsEU 835,000 individuals
HRQL in selective IgA deficiency
More infections, autoimmune disorders, allergic diseases
SIgAD32 individuals - 13 women, 19 men; mean age 48 years. Detected when screening blood donors and blood samples analysed at the University Hospital of Iceland. The blood from the 32 individuals was reanalysed to confirm inclusion criteria of S-IgA level <0.07g/L and no IgG subclass deficiency.Not under care, not informed
HRQL in selective IgA deficiency
SIgAD32 individuals - 13 women, 19 men; mean age 48 years. Detected when screening blood donors and blood samples analysed at the University Hospital of Iceland. The blood from the 32 individuals was reanalysed to confirm inclusion criteria of S-IgA level <0.07g/L and no IgG subclass deficiency.Not under care, not informed
Controls63 controls - 29 women, 34 men; mean age 50 years. A total of 96 age- and gender-matched controls were randomly selected from the Icelandic National Registry (1 SIgAD: 3 control individuals). 63 (66%) participated. All controls showed normal Ig levels.
HRQL in selective IgA deficiency
HRQL in selective IgA deficiency
No exclusion criteria for the individuals in the control group were set up
- thus, the controls might suffer from other chronic diseases/conditions and thereby better reflect the ordinary population.
HRQL in selective IgA deficiency
No exclusion criteria for the individuals in the control group were set up
- thus, the controls might suffer from other chronic diseases/conditions and thereby better reflect the ordinary population.
Unique study – first time in PID researchhistory that individuals with PID are compared to matched controls.
HRQL in selective IgA deficiency
Methods for HRQL and clinical status- Health and disease questionnaire (122 items) HRQLHRQL+CS+CS- SF-36 HRQL (36 items) HRQLHRQL- Infection-HRQL questionnaire (9 items) HRQLHRQL- Standardized interview by MD- Extensive physical examination- Lung function tests - Laboratory assessments- Skin prick tests
HRQL in selective IgA deficiency
Results clinical status – baseline (SIgAD vs controls)
We found that the SIgAD individuals significantly more often suffered from:
- upper- and lower respiratory tract infections
- autoimmune diseases
- allergies
- skin and nail-fungal infections
ESID Poster, 2012
HRQL in selective IgA deficiency
Remember the 30/70% slide?
ESID Poster, 2012
HRQL in selective IgA deficiency
Remember the 30/70% slide?Total disease burden when compared to controls
ESID Poster, 2012
HRQL in selective IgA deficiency
Remember the 30/70% slide?Total disease burden when compared to controls
ESID Poster, 2012
HRQL in selective IgA deficiency
Methods – 3 questionnaires for HRQL
SIgAD: Baseline 6 months 12 months
Controls: Baseline
HRQL in selective IgA deficiency
Results HRQL – baseline (SIgAD vs controls)
SF-36 results indicated poorer HRQL although not reaching statistical significances for the subscales (statistical power?)
Significantly increased fear of getting infected (p<0.01)
Correlation between fear of getting infection and physical health (p<0.01)
Significantly more SIgAD on long-term sick leave (p<0.05)
Baseline
Poor HRQL
12 months follow-up
6 months follow-up
Good HRQL Good HRQL
HRQL in selective IgA deficiency
Results – over time SIgAD
Baseline(dx)
Poor HRQL
12 months follow-up
6 months follow-up
Good HRQL Good HRQL
EducationTraining Home-therapy
HRQL in selective IgA deficiency
Results – over time SIgAD
Period of mixed feelings?
Risk factors for poor HRQLFactors Jörgensen
et al., ongoing
Age
Gender
Infections >4 organs
Number of infections/number of antibiotic treatments last 12 months
x
S-IgG level
Frequency of IVIG therapy
Type of diagnose
Home (instead of hospital)
Joint/muscle/skeletal x
No of any daily medication x
Allergic rhinoconjuctivitis x
Anxiety/insomnia x
Specific occurrence of stress last 2-3 months
Hospitalization last year
Unemployment
Smoking
Risk factors for poor HRQLFactors Jörgensen
et al., ongoing
Sigstad et al.,2005
Gardulf et al., 2008
Seeborg et al., ESID poster
Age x x (at dx)
Gender x
Infections >4 organs x
Number of infections/number of antibiotic treatments last 12 months
x x
S-IgG level x
Frequency of IVIG therapy x
Type of diagnose x
Home (instead of hospital) x
Joint/muscle/skeletal x x
No of any daily medication x
Allergic rhinoconjuctivitis x
Anxiety/insomnia x
Specific occurrence of stress last 2-3 months x
Hospitalization last year x
Unemployment x
Smoking x
HRQL in selective IgA deficiency
“What’s in it for me as a nurse?”
From a methodological point of view
The study highlights the importance to use matched controls in PID clinical research to better detect unexpected clinical manifestations, e.g. fungal infections for further research, and differences in HRQL.
Need to agree upon a basic “kit” of instruments/methods for international collaborations and comparisons betweencountries and patient groups.
HRQL in selective IgA deficiency
“What’s in it for me as a nurse?”
From a clinical point of view
SIgA is very common
Important to detect them as they have many health problems and are at risk for poor HRQL
Nursing interventions possible to prevent a worsening HRQL and/or to treat health problems
HRQL in selective IgA deficiency
“What’s in it for me as a nurse?”
From a clinical point of view
Aware of risk factors – more vulnerable subgroups?
Decreased HRQL for a period after being told about the PID- important for the nurse to know and to adapt educational/training based on this knowledge
HRQL in selective IgA deficiency
“What’s in it for me as a nurse?”
From a clinical point of view
Need for IgG therapy in some SIgAD individuals?
HRQL in selective IgA deficiency
Thank you !
Heimir & Pjakkur Màni (Moon)