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Immunological studies in the hyper-immunoglobulin D syndrome
A Haraldsson1, C M Weemaes, A W De Boer
1Department of Pediatrics, University Hospital Nijmegen, The Netherlands.
Journal of Clinical Immunology
|November 1, 1992
Summary
Hyper-immunoglobulin D syndrome (hyper-IgD syndrome) can affect multiple immunoglobulins, including elevated IgG3 and IgA, and decreased IgM. The IgD light-chain ratio is also disturbed in patients.
Area of Science:
- Immunology
- Genetics
Background:
- Hyper-immunoglobulin D syndrome (hyper-IgD syndrome) is a rare genetic autoinflammatory disorder.
- Characterized by recurrent fevers and elevated serum IgD levels.
Purpose of the Study:
- To investigate the longitudinal changes in immunoglobulin levels in patients with hyper-IgD syndrome.
- To explore the relationship between clinical symptoms and immunological markers.
Main Methods:
- Longitudinal follow-up of five hyper-IgD syndrome patients over 3-8 years.
- Measurement of serum IgG3, IgM, IgA, and IgD kappa/lambda ratios.
Main Results:
- All patients exhibited high serum IgG3 levels.
- IgM decreased, while IgA was elevated in most patients during follow-up.
- The IgD kappa/lambda ratio was raised in all assessed patients, despite normal total light-chain ratios.
Conclusions:
- Hyper-IgD syndrome impacts multiple immunoglobulin classes beyond IgD.
- Disturbances in IgD light-chain ratios are characteristic of the condition.
- Clinical manifestations may precede or coincide with immunological changes, suggesting a common underlying factor.