hyper-IgD syndrome
MIM.260920 12q24
HIDS
HIDS is a periodic-fever syndrome caused by a genetic defect in mevalonate kinase (MVK).
Despite a frequent, often persistent, and vigorous acute-phase response similar to that seen in patients with other periodic-fever syndromes, amyloidosis does not develop in patients with HIDS. This intriguing finding suggests that mevalonate kinase deficiency may provide protection against amyloidosis.
Etiology
The hyper-IgD syndrome is caused by mutations in the gene encoding mevalonate kinase (MVK) (MIM.251170).