Other Names for this Disease
- Hyper IgD syndrome
- Hyperimmunoglobulinemia D and periodic fever syndrome
- Periodic fever Dutch type
What are the signs and symptoms of hyper IgD syndrome?
What causes hyper IgD syndrome?
Is hyper IgD syndrome inherited?
How might hyper IgD syndrome be treated?
What is the prognosis for individuals with hyper IgD syndrome?
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.
Consultations with the following specialists may be helpful: dermatologist, rheumatologist, and infectious disease specialist (to evaluate periodic fever).
- Hyper IgD Syndrome. National Organization for Rare Disorders (NORD). 2006; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/1169/viewAbstract. Accessed 3/5/2013.
- Shinawi M, Scaglia F. Hereditary Periodic Fever Syndromes. Medscape Reference. 2013; http://emedicine.medscape.com/article/952254-overview. Accessed 3/5/2013.
- Hyper-IgD Syndrome. Online Mendelian Inheritance in Man (OMIM). 2009; http://omim.org/entry/260920. Accessed 3/5/2013.
- Frenkel J, Simon A. Hyperimmunoglobulinemia D with recurrent fever. Orphanet. 2011; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=343. Accessed 3/5/2013.