Diagnosis of hyperimmunoglobulin D syndrome in the patient with a suspected periodic fever syndrome*

HIDS: hyperimmunoglobulin D syndrome; MKD: mevalonate kinase deficiency; MA: mevalonic aciduria; IgD: immunoglobulin D; MVK: mevalonate kinase; FMF: familial Mediterranean fever; CAPS: cryopyrin-associated periodic syndrome; TRAPS: tumor necrosis factor alpha receptor-1 associated periodic syndrome; PFAPA: periodic fever with aphthous stomatitis, pharyngitis, and adenitis.
* A periodic fever syndrome should be suspected in a patient with childhood to early adulthood onset of fevers that recur over months to years in the absence of infection or malignancy.
¶ The Eurofever Classification Criteria scoring system screens for the most common periodic fever syndromes, including FMF, CAPS, and TRAPS. It does not include PFAPA. Refer to appropriate topic reviews for details on clinical manifestations of each periodic fever syndrome.
Δ Clinical features consistent with variant HIDS include onset at ≥5 years of age, fever duration >14 days, and lack of associated joint pain.