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Clinical and Diagnostic Laboratory Immunology, January 2001, p. 58-61, Vol. 8, No. 1
1071-412X/01/$04.00+0   DOI: 10.1128/CDLI.8.1.58-61.2001
Copyright © 2001, American Society for Microbiology. All rights reserved.

Hyper-Immunoglobulin A in the Hyperimmunoglobulinemia D Syndrome

Ina S. Klasen,1,* Joop H. C. Göertz,1 Gertrude A. S. van de Wiel,1 Corry M. R. Weemaes,2 Jos W. M. van der Meer,3 and Joost P. H. Drenth3

Department of Clinical Chemistry,1 Department of Pediatrics,2 and Department of Internal Medicine,3 University Medical Center Nijmegen, Nijmegen, The Netherlands

Received 30 June 2000/Returned for modification 4 August 2000/Accepted 6 October 2000

The hyperimmunoglobulinemia D syndrome (HIDS) is an autosomal recessive disorder characterized by recurrent febrile attacks with abdominal, articular, and skin manifestations. Apart from elevated immunoglobulin D (IgD) levels (>100 IU/ml), there are high IgA levels in the majority of cases. Mutations in the gene encoding mevalonate kinase constitute the molecular defect in HIDS. The cause of elevated IgA concentrations in HIDS patients remains to be elucidated. We studied the hyper-IgA response in serum of a group of HIDS patients. Elevated IgA concentrations result from increased IgA1 concentrations. IgA and IgA1 concentrations correlated significantly with IgD concentrations, and levels of IgA polymers were significantly higher than the levels in healthy donors. These results indicate a continuous, presumably systemic, stimulation of IgA in HIDS patients.

* Corresponding author. Mailing address: 564 Department of Clinical Chemistry, University Medical Center Nijmegen, P. O. Box 9101, 6500 HB Nijmegen, The Netherlands. Phone: 31.243614777. Fax: 31.243541743. E-mail: I.Klasen{at}ckcl.azn.nl.

Clinical and Diagnostic Laboratory Immunology, January 2001, p. 58-61, Vol. 8, No. 1
1071-412X/01/$04.00+0   DOI: 10.1128/CDLI.8.1.58-61.2001
Copyright © 2001, American Society for Microbiology. All rights reserved.


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