Hyper-IgD syndrome (HIDS) is due to mutations of the gene coding for mevalonate kinase, an enzyme that has a pivotal role in the synthesis of isoprenoids and cholesterol.1 So far, there is no consensus about how HIDS should be treated. Here we report our experience with a child with HIDS treated with different drug regimens.

The child was born to healthy, unrelated Italian parents. He came to our attention because of periodic fever spikes, which occurred every 20–30 days. During fever flare ups, he usually developed chills, arthralgias without arthritis, malaise, and abdominal pain with diarrhoea. Severe leucocytosis (up to 39 × 10⁹/l) and acute phase reactant positivity (C reactive protein 2.9 mg/l; normal values <4 mg/l) were also detected. An abdominal echo …