Features of hyperimmunoglobulin D syndrome (HIDS)

General

High IgD (>14 mg/dL), measured on 2 occasions, at least 1 month apart

Elevated IgA (>260 mg/dL)

Recurrent attacks

Mutations in MVK gene (classic HIDS)

Decreased MVK activity (classic HIDS)

During Attacks

Elevated inflammatory markers (ESR, CRP, SAA, and leukocytosis)

Abrupt onset of fever (>38.5°C or 101.3°F)

Lymphadenopathy (predominantly cervical)

Abdominal distress (vomiting, diarrhea, pain)

Skin manifestations (varying erythematous macules, papules, petichiae, and purpura)

Arthralgias/arthritis

Splenomegaly

Mildly elevated urinary mevalonic acid concentration (classic HIDS)

IgD: immunoglobulin D; IgA: immunoglobulin A; MVK: mevalonate kinase; ESR: erythrocyte sedimentation rate; CRP: C-reactive protein; SAA: serum amyloid A.

Data from: Drenth JP, Haagsma CJ, van der Meer JW. Medicine (Baltimore) 1994; 73:133 and Drenth JP, van Deuren M, van der Ven-Jongekrijg J, et al. Blood 1995; 85:3586.

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Features of hyperimmunoglobulin D syndrome (HIDS)