Neuropediatrics 2007; 38(6): 269-275
DOI: 10.1055/s-2008-1065352
Original Article

© Georg Thieme Verlag KG Stuttgart · New York

Plasmacytoid Dendritic Cells and Interferon-alpha in Aicardi-Goutières Syndrome

J. T. van Heteren 1 , 2 , M. S. van der Knaap 3 , B. W. Poll The 1 , T. W. Kuijpers 1
  • 1Emma Children's Hospital, Academic Medical Center (AMC), Amsterdam, The Netherlands
  • 2AMC, Department of Experimental Immunology, Amsterdam, The Netherlands
  • 3VU University Medical Center (VUMC), Department of Child Neurology, Amsterdam, The Netherlands
Further Information

Publication History

received 15.05.2007

accepted 26.02.2008

Publication Date:
06 May 2008 (online)


In Aicardi-Goutières syndrome (AGS), as in systemic lupus erythematosus (SLE) and Sjögren's syndrome, an increased level of interferon alpha (IFN-α) is involved in the pathogenesis of the disease. In SLE and Sjögren's syndrome, cytokine production originates in plasmacytoid dendritic cells (pDCs) under the influence of immune complexes formed by DNA and RNA from improperly removed apoptotic or necrotic cells, together with IgG autoantibodies. We studied the role of soluble factors in the serum or cerebrospinal fluid (CSF) of AGS patients and their capacity to stimulate pDCs to produce IFN-α. Our findings show that, in contrast to SLE, there is no decrease in the number of circulating pDCs in AGS patients. Secondly, unlike the autoantibodies in the serum of patients with SLE or Sjögren's syndrome, there is no increased frequency of antinuclear antibodies (ANA) or other soluble factors inducing IFN-α from pDCs. These data indicate that the origin of IFN-α in AGS is different from that in the autoimmune diseases tested.



J.T. van Heteren

Department of Experimental Immunology

Academic Medical Center

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