Non x-linked hyper-IgM syndrome with systemic lupus erythematosus

J. Arai, M. Yasukawa, K. Takada, Y. Tange, O. Saiki, T. Horiuchi, T. Tamai, S. Fujita

Research output: Contribution to journalArticlepeer-review

7 Citations (Scopus)


A 24-'year-old woman had suffered from recurrent bacterial infections and clinical manifestations of systemic lupus erythematosus (SLE). Laboratory findings disclosed an elevated level of serum IgM, markedly decreased IgG, IgA, IgD and IgE levels, and low levels of serum complement. Both the CD40 and CD40 ligands appeared to be normally expressed. Assays of in vitro immunoglobulin production by lymphocytes showed that IgM was produced normally and that IgE but not IgG or IgA production was rescued by signalling through CD40 on B cells. The proliferative response of lymphocytes to phobol ester was some impairment of signal transduction in the patient's lymphocytes.

Original languageEnglish
Pages (from-to)84-86
Number of pages3
JournalClinical and experimental rheumatology
Issue number1
Publication statusPublished - 1998

All Science Journal Classification (ASJC) codes

  • Rheumatology
  • Immunology and Allergy
  • Immunology


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