Autoimmunity in X-linked agammaglobulinemia: Kawasaki disease and review of the literature

Expert Rev Clin Immunol. 2012 Feb;8(2):155-9. doi: 10.1586/eci.11.94.

Abstract

Although autoimmunity phenotype is surprisingly common in patients with different types of primary antibody deficiency, it is much less frequent in X-linked agammaglobulinemia (XLA). Herein, we report on a 15-month-old boy with XLA who also suffered from Kawasaki disease. The current case presentation is the first report of an association between Kawasaki disease and XLA. XLA could be considered as a special opportunity to understand autoimmunity in the near absence of immunoglobulins.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Agammaglobulinemia* / complications
  • Agammaglobulinemia* / drug therapy
  • Agammaglobulinemia* / genetics
  • Agammaglobulinemia* / immunology
  • Agammaglobulinemia* / pathology
  • Genetic Diseases, X-Linked* / complications
  • Genetic Diseases, X-Linked* / drug therapy
  • Genetic Diseases, X-Linked* / genetics
  • Genetic Diseases, X-Linked* / immunology
  • Genetic Diseases, X-Linked* / pathology
  • Humans
  • Male
  • Mucocutaneous Lymph Node Syndrome* / complications
  • Mucocutaneous Lymph Node Syndrome* / genetics
  • Mucocutaneous Lymph Node Syndrome* / immunology
  • Mucocutaneous Lymph Node Syndrome* / pathology