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Genetic and Rare Diseases Information Center (GARD)

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Hyper-IgD syndrome

Other Names for this Disease
  • Hyper IgD syndrome
  • Hyperimmunoglobulinemia D and periodic fever syndrome
  • Periodic fever Dutch type
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How might hyper IgD syndrome be treated?

There is no cure for hyper IgD syndrome and currently no established treatment.  Management is focused on supportive care. Some patients have responded to high-dose prednisone. Simvastatin, Anakinria (an IL-1 receptor antagonist) and TNF inhibitors have recently shown some success in controlling inflammatory attacks.[1][2]  

Consultations with the following specialists may be helpful: dermatologist, rheumatologist, and infectious disease specialist (to evaluate periodic fever).[1] 

Last updated: 3/5/2013

  1. Shinawi M, Scaglia F. Hereditary Periodic Fever Syndromes. Medscape Reference. 2013; Accessed 3/5/2013.
  2. Frenkel J, Simon A. Hyperimmunoglobulinemia D with recurrent fever. Orphanet. 2011; Accessed 3/5/2013.

Clinical Trials & Research for this Disease

  • lists trials that are studying or have studied Hyper-IgD syndrome. Click on the link to go to to read descriptions of these studies.