Nijmegen periodic fever research group

Hyper-IgD and periodic fever syndrome (HIDS)

Diagnostic indicators

At attacks

> Abrupt onset of fever (≥38.5°C)

> Recurrent attacks from early youth

> Lymphadenopathy (especially cervical)

> Abdominal distress: vomiting, diarrhoea, pain

> Skin manifestations (especially erythematous macules and papules)

> Arthralgias / Arthritis

> Splenomegaly

> Acute phase response: elevated ESR and leukocytosis

> Classic HIDS: elevated urinary concentration mevalonic acid

 

Constantly present

> Elevated IgD (≥ 100 U/mL) measured at 2 occasions with at least one month apart

> Elevated IgA (≥2.6 g/L)

> Classic HIDS: mutations in mevalonate kinase gene

> Classic HIDS: decreased mevalonate kinase enzyme activity

 

(Extremely) high serum concentrations of IgD are characteristic but not obligatory.

 

 

 

Last update: February 8, 2004