Hyper IgD syndrome

Reference work entry
DOI: https://doi.org/10.1007/3-540-29662-X_1281


The hyper-IgD syndrome (HIDS) is characterized by attacks of fevers and chills, lasting four to six days. Other clinical features include cervical lymphadenopathy, abdominal pain, nausea, vomiting, diarrhea, rash, headache, arthralgias, arthritis, and elevated serum IgD levels. These episodes may be triggered by surgery, trauma, vaccinations, or other stressors. Patients are relatively asymptomatic between episodes, usually four to six weeks apart. The syndrome develops within the first year of life, and some children do not have elevated IgD levels. Laboratory features during an attack reveal elevated C-reactive protein, leukocytosis, and elevated serum amyloid A.

HIDS is an autosomal recessive disease, most common in Caucasians. Mutations in the gene for mevalonate kinase, an enzyme in cholesterol metabolism, have been identified in these patients. Mevalonate kinase activity and serum cholesterol are reduced in patients with the hyper-IgD syndrome. During febrile attacks,...

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