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Association Between IgA Deficiency & Other Autoimmune Conditions: A Population-Based Matched Cohort Study

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To examine autoimmune disorders in patients with IgA deficiency compared with the general population.


Nationwide prospective population-based cohort study. Through six university hospitals in Sweden we identified 2100 individuals with IgA deficiency (IgA levels < .07 g/L) diagnosed between 1980 and 2011. Each patient with IgA deficiency was matched on age, sex, place of residence, and year of diagnosis with up to 10 general population controls (n = 18,653). Data on nine autoimmune disorders were retrieved from the Swedish National Patient Register (including inpatient and non-primary outpatient care). Autoimmune disorders were defined as having at least two visits listing the relevant international classification of disease (ICD) code as main diagnosis. Prevalences and prevalence ratios (PRs) were calculated.


Individuals with IgA deficiency more often had celiac disease (6.7 % vs. 0.19 % in controls) and type 1 diabetes (5.9 % vs. 0.57 %) corresponding to a 35-fold higher PR for celiac disease and 10-fold higher for type 1 diabetes. Also for the other autoimmune diseases did we see statistically significantly elevated prevalences and PRs (juvenile idiopathic arthritis (0.76 % vs. 0.09 % in controls, PR = 8.9), systemic lupus erythematosus (0.57 % vs. 0.06 %; PR = 8.9), inflammatory bowel disease (3.9 % vs. 0.81 %; PR = 5.0; specifically Crohn’s disease (2.4 % vs. 0.42 %; PR = 5.7) and ulcerative colitis (1.7 % vs. 0.46 %; PR = 3.9)), hypothyreosis (0.76 % vs. 0.16 %; PR = 4.6), rheumatoid arthritis (2.2 % vs. 0.50 %; PR = 4.5), and hyperthyreosis (1.7 % vs. 0.43 %; PR = 3.9), but not with myasthenia gravis (0.05 % vs. 0.02 %; PR = 3.0).


Individuals with IgA deficiency have a higher prevalence of several other autoimmune disorders.

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Prevalence ratio


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Conflict of Interest

The authors (JFL, MN, LH) declare that they have no conflicts of interest relevant to the contents of this manuscript.

Details of Contributors

Dr Ludvigsson and Dr Neovius had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis.

Study concept and design: Ludvigsson

Acquisition of data: Hammarström

Drafting of the manuscript: Ludvigsson, Neovius

Critical revision of the manuscript for important intellectual content: Hammarström, Ludvigsson, Neovius

Statistical analysis: Ludvigsson, Neovius

Obtained funding: Hammarström

Study supervision: Ludvigsson, Hammarström

Ethical Approval

This project (2011/69-31/3) was approved by the Regional Ethical Review Board in Stockholm on Feb 23, 2011. This was a register-based study and therefore all data were anonymised prior to analysis, and we were not allowed to contact the patients.


JFL was supported by grants from the Swedish Society of Medicine, and the Swedish Research Council; MN: None; LH: Swedish Research Council.

Statement of Independence of Researchers from Funders

No person representing the funding sources read or commented on any version of the manuscript.

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Corresponding author

Correspondence to Jonas F. Ludvigsson.



Table II Prevalence of autoimmune diseases in patients with diagnosed IgA deficiency and matched general population controls

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Ludvigsson, J.F., Neovius, M. & Hammarström, L. Association Between IgA Deficiency & Other Autoimmune Conditions: A Population-Based Matched Cohort Study. J Clin Immunol 34, 444–451 (2014).

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