HIDS is an autosomal recessive disorder caused by mutations in the MVK gene. The disorder usually presents in childhood, often before one year of age. Attacks typically last from three days to a week, often with a one to two month interval between them. Attacks are characterized by high spiking fever,abdominal pain, vomiting, diarrhea, headache, arthralgias, and swollen, tender lymph nodes. Red macules of the skin and aphthous ulcers may also be observed. Many patients have nondestructive recurrent arthritis, especially of the larger joints, and many younger patients exhibit splenomegaly. Constitutively elevated serum IgD levels (> 100 U/mL; 14.1 mg/dL) are observed in the majority of patients but are not required for the diagnosis. Most patients are not responsive to colchicine, but treatment with corticosteroids, anakinra, and etanercept may be effective.