Publication: An Adolescent with Hyperimmunoglobulinemia D and Periodic Fever Syndrome Responding to Simvastatin Treatment
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Date
2011-06-15
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TURKISH LEAGUE AGAINST RHEUMATISM
Abstract
The hyperimmunoglobulinemia D and periodic fever syndrome is proposed to be caused by a defect in the activity of mevalonate kinase enzyme which is involved in cholesterol and non-sterol isoprenoid biosynthesis. This autosomal recessive inherited auto-inflammatory syndrome is characterized by recurrent fever attacks, abdominal pain, lymphadenopathy, skin lesions and joint involvement. In this article, we present our therapeutic approach with the hypolipidemic agent, simvastatin, in a 12-year-old boy followed up with a diagnosis of hyperimmunoglobulinemia D and periodic fever syndrome. Simvastatin treatment of an adolescent with hyperimmunoglobulinemia D and periodic fever syndrome unresponsive to anti-inflammatory strategies has resulted in a favorable outcome. This treatment is thought to reduce the recurrent fever attacks by reducing the mevalonic acid increase or isopreniod shortage.
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Keywords
Hyperimmunoglobulinemia D, periodic fever syndrome, simvastatin, MEVALONATE KINASE-DEFICIENCY, HYPER-IGD, ISOPRENOID BIOSYNTHESIS, FOLLOW-UP, MUTATIONS, ATTACKS, SERIES, CELLS