Immunoglobulin D (IgD)

Immunology

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Description

Hyper IgD (HIDS) is a rare syndrome with features of recurrent bouts of fever, lymphadenitis and occasionally arthritis. The majority, over 70%, of patients have an IgD level of >100 KU/L and therefore over 20% of patients have an IgD level within the reference range. The underlying genetic defect is a mutation of the mevalonate kinase (MVK) gene leading to mevalonate kinase deficiency (MKD) [1]. High IgD levels can occur in other conditions with a normal MVK genotype [1].

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Indications

Hyper IgD Syndrome. Periodic Fever Syndrome. Autoinflammatory syndrome.

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Sample Type

2mL Serum (Gel 5mL Yellow tube). Requests from outside Sheffield: Transport at ambient temperature via Royal Mail or Courier.

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Reference Range

2 - 100 KU/L.Reference range established in house.

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Turnaround Time

Within 2 weeks

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Testing Frequency

As required

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References

Ammouri W, et al. Diagnostic value of serum immunoglobulinaemia D level in patients with a clinical suspicion of hyper IgD syndrome. Rheumatology 2007. 46:1597-1600. [Ref 1]Simon A, et al. Molecular analysis of the mevalonate kinase gene in a cohort of patients with the hyper IgD and Periodic fever syndrome: its application as a diagnostic tool. Ann. Intern. Med. 2001 135:338-343.

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Please note: the above information is subject to change and we endeavour to keep this website up to date wherever necessary.