Hyperimmunoglobulinemia D and periodic fever syndrome (HIDS) in a child with normal serum IgD, but increased serum IgA concentration

J Pediatr. 2003 Jul;143(1):127-9. doi: 10.1016/S0022-3476(03)00212-9.


This report describes a boy with hyperimmunoglobulinemia D and periodic fever syndrome (HIDS). The serum IgD level was normal, but the serum IgA concentration was markedly elevated. In addition, he had a history of orchitis on two occasions, a previously unreported manifestation of HIDS. This report expands the clinical and laboratory features associated with HIDS and serves to emphasize that a normal serum IgD level does not exclude the diagnosis of HIDS.

Publication types

  • Case Reports

MeSH terms

  • Anti-Inflammatory Agents / therapeutic use
  • Child
  • DNA Mutational Analysis
  • Familial Mediterranean Fever / blood*
  • Familial Mediterranean Fever / drug therapy
  • Familial Mediterranean Fever / genetics
  • Gene Expression / genetics
  • Humans
  • Immunoglobulin A / blood*
  • Immunoglobulin D / blood*
  • Male
  • Phosphotransferases (Alcohol Group Acceptor) / genetics
  • Point Mutation / genetics
  • Prednisolone / therapeutic use


  • Anti-Inflammatory Agents
  • Immunoglobulin A
  • Immunoglobulin D
  • Prednisolone
  • Phosphotransferases (Alcohol Group Acceptor)
  • mevalonate kinase